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Sample records for hearing gene prestin

  1. The hearing gene Prestin reunites echolocating bats

    PubMed Central

    Li, Gang; Wang, Jinhong; Rossiter, Stephen J.; Jones, Gareth; Cotton, James A.; Zhang, Shuyi

    2008-01-01

    The remarkable high-frequency sensitivity and selectivity of the mammalian auditory system has been attributed to the evolution of mechanical amplification, in which sound waves are amplified by outer hair cells in the cochlea. This process is driven by the recently discovered protein prestin, encoded by the gene Prestin. Echolocating bats use ultrasound for orientation and hunting and possess the highest frequency hearing of all mammals. To test for the involvement of Prestin in the evolution of bat echolocation, we sequenced the coding region in echolocating and nonecholocating species. The resulting putative gene tree showed strong support for a monophyletic assemblage of echolocating species, conflicting with the species phylogeny in which echolocators are paraphyletic. We reject the possibilities that this conflict arises from either gene duplication and loss or relaxed selection in nonecholocating fruit bats. Instead, we hypothesize that the putative gene tree reflects convergence at stretches of functional importance. Convergence is supported by the recovery of the species tree from alignments of hydrophobic transmembrane domains, and the putative gene tree from the intra- and extracellular domains. We also found evidence that Prestin has undergone Darwinian selection associated with the evolution of specialized constant-frequency echolocation, which is characterized by sharp auditory tuning. Our study of a hearing gene in bats strongly implicates Prestin in the evolution of echolocation, and suggests independent evolution of high-frequency hearing in bats. These results highlight the potential problems of extracting phylogenetic signals from functional genes that may be prone to convergence. PMID:18776049

  2. Hearing aid for vertebrates via multiple episodic adaptive events on prestin genes.

    PubMed

    Liu, Zhen; Li, Gong-Hua; Huang, Jing-Fei; Murphy, Robert W; Shi, Peng

    2012-09-01

    Auditory detection is essential for survival and reproduction of vertebrates, yet the genetic changes underlying the evolution and diversity of hearing are poorly documented. Recent discoveries concerning prestin, which is responsible for cochlear amplification by electromotility, provide an opportunity to redress this situation. We identify prestin genes from the genomes of 14 vertebrates, including three fishes, one amphibian, one lizard, one bird, and eight mammals. An evolutionary analysis of these sequences and 34 previously known prestin genes reveals for the first time that this hearing gene was under positive selection in the most recent common ancestor (MRCA) of tetrapods. This discovery might document the genetic basis of enhanced high sound sensibility in tetrapods. An investigation of the adaptive gain and evolution of electromotility, an important evolutionary innovation for the highest hearing ability of mammals, detects evidence for positive selections on the MRCA of mammals, therians, and placentals, respectively. It is suggested that electromotility determined by prestin might initially appear in the MRCA of mammals, and its functional improvements might occur in the MRCA of therian and placental mammals. Our patch clamp experiments further support this hypothesis, revealing the functional divergence of voltage-dependent nonlinear capacitance of prestin from platypus, opossum, and gerbil. Moreover, structure-based cdocking analyses detect positively selected amino acids in the MRCA of placental mammals that are key residues in sulfate anion transport. This study provides new insights into the adaptation and functional diversity of hearing sensitivity in vertebrates by evolutionary and functional analysis of the hearing gene prestin. PMID:22416033

  3. Parallel sites implicate functional convergence of the hearing gene prestin among echolocating mammals.

    PubMed

    Liu, Zhen; Qi, Fei-Yan; Zhou, Xin; Ren, Hai-Qing; Shi, Peng

    2014-09-01

    Echolocation is a sensory system whereby certain mammals navigate and forage using sound waves, usually in environments where visibility is limited. Curiously, echolocation has evolved independently in bats and whales, which occupy entirely different environments. Based on this phenotypic convergence, recent studies identified several echolocation-related genes with parallel sites at the protein sequence level among different echolocating mammals, and among these, prestin seems the most promising. Although previous studies analyzed the evolutionary mechanism of prestin, the functional roles of the parallel sites in the evolution of mammalian echolocation are not clear. By functional assays, we show that a key parameter of prestin function, 1/α, is increased in all echolocating mammals and that the N7T parallel substitution accounted for this functional convergence. Moreover, another parameter, V1/2, was shifted toward the depolarization direction in a toothed whale, the bottlenose dolphin (Tursiops truncatus) and a constant-frequency (CF) bat, the Stoliczka's trident bat (Aselliscus stoliczkanus). The parallel site of I384T between toothed whales and CF bats was responsible for this functional convergence. Furthermore, the two parameters (1/α and V1/2) were correlated with mammalian high-frequency hearing, suggesting that the convergent changes of the prestin function in echolocating mammals may play important roles in mammalian echolocation. To our knowledge, these findings present the functional patterns of echolocation-related genes in echolocating mammals for the first time and rigorously demonstrate adaptive parallel evolution at the protein sequence level, paving the way to insights into the molecular mechanism underlying mammalian echolocation. PMID:24951728

  4. Prestin-Dependence of Outer Hair Cell Survival and Partial Rescue of Outer Hair Cell Loss in PrestinV499G/Y501H Knockin Mice.

    PubMed

    Cheatham, Mary Ann; Edge, Roxanne M; Homma, Kazuaki; Leserman, Emily L; Dallos, Peter; Zheng, Jing

    2015-01-01

    A knockin (KI) mouse expressing mutated prestinV499G/Y501H (499 prestin) was created to study cochlear amplification. Recordings from isolated outer hair cells (OHC) in this mutant showed vastly reduced electromotility and, as a consequence, reduced hearing sensitivity. Although 499 prestin OHCs were normal in stiffness and longer than OHCs lacking prestin, accelerated OHC death was unexpectedly observed relative to that documented in prestin knockout (KO) mice. These observations imply an additional role of prestin in OHC maintenance besides its known requirement for mammalian cochlear amplification. In order to gain mechanistic insights into prestin-associated OHC loss, we implemented several interventions to improve survival. First, 499 prestin KI's were backcrossed to Bak KO mice, which lack the mitochondrial pro-apoptotic gene Bak. Because oxidative stress is implicated in OHC death, another group of 499 prestin KI mice was fed the antioxidant diet, Protandim. 499 KI mice were also backcrossed onto the FVB murine strain, which retains excellent high-frequency hearing well into adulthood, to reduce the compounding effect of age-related hearing loss associated with the original 499 prestin KIs. Finally, a compound heterozygous (chet) mouse expressing one copy of 499 prestin and one copy of KO prestin was also created to reduce quantities of 499 prestin protein. Results show reduction in OHC death in chets, and in 499 prestin KIs on the FVB background, but only a slight improvement in OHC survival for mice receiving Protandim. We also report that improved OHC survival in 499 prestin KIs had little effect on hearing phenotype, reaffirming the original contention about the essential role of prestin's motor function in cochlear amplification. PMID:26682723

  5. Prestin-Dependence of Outer Hair Cell Survival and Partial Rescue of Outer Hair Cell Loss in PrestinV499G/Y501H Knockin Mice

    PubMed Central

    Cheatham, Mary Ann; Edge, Roxanne M.; Homma, Kazuaki; Leserman, Emily L.; Dallos, Peter; Zheng, Jing

    2015-01-01

    A knockin (KI) mouse expressing mutated prestinV499G/Y501H (499 prestin) was created to study cochlear amplification. Recordings from isolated outer hair cells (OHC) in this mutant showed vastly reduced electromotility and, as a consequence, reduced hearing sensitivity. Although 499 prestin OHCs were normal in stiffness and longer than OHCs lacking prestin, accelerated OHC death was unexpectedly observed relative to that documented in prestin knockout (KO) mice. These observations imply an additional role of prestin in OHC maintenance besides its known requirement for mammalian cochlear amplification. In order to gain mechanistic insights into prestin-associated OHC loss, we implemented several interventions to improve survival. First, 499 prestin KI’s were backcrossed to Bak KO mice, which lack the mitochondrial pro-apoptotic gene Bak. Because oxidative stress is implicated in OHC death, another group of 499 prestin KI mice was fed the antioxidant diet, Protandim. 499 KI mice were also backcrossed onto the FVB murine strain, which retains excellent high-frequency hearing well into adulthood, to reduce the compounding effect of age-related hearing loss associated with the original 499 prestin KIs. Finally, a compound heterozygous (chet) mouse expressing one copy of 499 prestin and one copy of KO prestin was also created to reduce quantities of 499 prestin protein. Results show reduction in OHC death in chets, and in 499 prestin KIs on the FVB background, but only a slight improvement in OHC survival for mice receiving Protandim. We also report that improved OHC survival in 499 prestin KIs had little effect on hearing phenotype, reaffirming the original contention about the essential role of prestin’s motor function in cochlear amplification. PMID:26682723

  6. Prestin is an anion transporter dispensable for mechanical feedback amplification in Drosophila hearing.

    PubMed

    Kavlie, Ryan G; Fritz, Janice L; Nies, Florian; Göpfert, Martin C; Oliver, Dominik; Albert, Joerg T; Eberl, Daniel F

    2015-01-01

    In mammals, the membrane-based protein Prestin confers unique electromotile properties to cochlear outer hair cells, which contribute to the cochlear amplifier. Like mammals, the ears of insects, such as those of Drosophila melanogaster, mechanically amplify sound stimuli and have also been reported to express Prestin homologs. To determine whether the D. melanogaster Prestin homolog (dpres) is required for auditory amplification, we generated and analyzed dpres mutant flies. We found that dpres is robustly expressed in the fly's antennal ear. However, dpres mutant flies show normal auditory nerve responses, and intact non-linear amplification. Thus we conclude that, in D. melanogaster, auditory amplification is independent of Prestin. This finding resonates with prior phylogenetic analyses, which suggest that the derived motor function of mammalian Prestin replaced, or amended, an ancestral transport function. Indeed, we show that dpres encodes a functional anion transporter. Interestingly, the acquired new motor function in the phylogenetic lineage leading to birds and mammals coincides with loss of the mechanotransducer channel NompC (=TRPN1), which has been shown to be required for auditory amplification in flies. The advent of Prestin (or loss of NompC, respectively) may thus mark an evolutionary transition from a transducer-based to a Prestin-based mechanism of auditory amplification. PMID:25412730

  7. Genes and Hearing Loss

    MedlinePlus

    ... Meeting Calendar Find an ENT Doctor Near You Genes and Hearing Loss Genes and Hearing Loss Patient ... mutation may only have dystopia canthorum. How Do Genes Work? Genes are a road map for the ...

  8. Reduction in Noise-Induced Functional Loss of the Cochleae in Mice with Pre-Existing Cochlear Dysfunction Due to Genetic Interference of Prestin

    PubMed Central

    Cai, Qunfeng; Wang, Bo; Coling, Donald; Zuo, Jian; Fang, Jie; Yang, Shiming; Vera, Krystal; Hu, Bo Hua

    2014-01-01

    Various cochlear pathologies, such as acoustic trauma, ototoxicity and age-related degeneration, cause hearing loss. These pre-existing hearing losses can alter cochlear responses to subsequent acoustic overstimulation. So far, the knowledge on the impacts of pre-existing hearing loss caused by genetic alteration of cochlear genes is limited. Prestin is the motor protein expressed exclusively in outer hair cells in the mammalian cochlea. This motor protein contributes to outer hair cell motility. At present, it is not clear how the interference of prestin function affects cochlear responses to acoustic overstimulation. To address this question, a genetic model of prestin dysfunction in mice was created by inserting an internal ribosome entry site (IRES)-CreERT2-FRT-Neo-FRT cassette into the prestin locus after the stop codon. Homozygous mice exhibit a threshold elevation of auditory brainstem responses with large individual variation. These mice also display a threshold elevation and a shift of the input/output function of the distortion product otoacoustic emission, suggesting a reduction in outer hair cell function. The disruption of prestin function reduces the threshold shifts caused by exposure to a loud noise at 120 dB (sound pressure level) for 1 h. This reduction is positively correlated with the level of pre-noise cochlear dysfunction and is accompanied by a reduced change in Cdh1 expression, suggesting a reduction in molecular responses to the acoustic overstimulation. Together, these results suggest that prestin interference reduces cochlear stress responses to acoustic overstimulation. PMID:25486270

  9. Prestin and the good vibrations

    PubMed Central

    Birke, Anna Sofia; Javelle, Arnaud

    2016-01-01

    In a recent paper published in the Biochemical Journal, Lolli et al. presented evidence that the C-terminal STAS (sulfate transporter and anti-sigma factor antagonist) domain of the motor protein prestin possesses an anion-binding site. This discovery might shed light on an aspect of the function of this mysterious and fascinating protein that is crucial for the human hearing system. PMID:27470595

  10. Prestin and the good vibrations.

    PubMed

    Birke, Anna Sofia; Javelle, Arnaud

    2016-08-01

    In a recent paper published in the Biochemical Journal, Lolli et al. presented evidence that the C-terminal STAS (sulfate transporter and anti-sigma factor antagonist) domain of the motor protein prestin possesses an anion-binding site. This discovery might shed light on an aspect of the function of this mysterious and fascinating protein that is crucial for the human hearing system. PMID:27470595

  11. Stable Expression of the Motor Protein Prestin in Chinese Hamster Ovary Cells

    NASA Astrophysics Data System (ADS)

    Iida, Koji; Konno, Kazuaki; Oshima, Takeshi; Tsumoto, Kouhei; Ikeda, Katsuhisa; Kumagai, Izumi; Kobayashi, Toshimitsu; Wada, Hiroshi

    Mammalian hearing sensitivity relies on a mechanical amplification mechanism involving the outer hair cells (OHCs), which rapidly alter their longitudinal length in response to changes in their membrane potential. The molecular basis of this mechanism is thought to be a motor protein embedded in the lateral membrane of the OHCs. Recently, this motor protein was identified and termed prestin. Since then, prestin has been researched intensively to elucidate the behavior of the OHCs. However, little progress in the study of prestin at the molecular level has been made because no method of obtaining an adequate amount of prestin has been established. In this study, therefore, an attempt was made to construct a stable expression system of prestin using Chinese hamster ovary (CHO) cells. The expression of prestin in the transfected CHO cells and the activity of prestin on CHO cells were confirmed by immunofluorescence and whole-cell patch-clamp measurements, respectively.

  12. Abnormal mRNA splicing but normal auditory brainstem response (ABR) in mice with the prestin (SLC26A5) IVS2-2A>G mutation.

    PubMed

    Zhang, Jian; Liu, Ziyi; Chang, Aoshuang; Fang, Jie; Men, Yuqin; Tian, Yong; Ouyang, Xiaomei; Yan, Denise; Zhang, Aizhen; Sun, Xiaoyang; Tang, Jie; Liu, Xuezhong; Zuo, Jian; Gao, Jiangang

    2016-08-01

    Prestin is critical to OHC somatic motility and hearing sensitivity in mammals. Several mutations of the human SLC26A5 gene have been associated with deafness. However, whether the IVS2-2A>G mutation in the human SLC26A5 gene causes deafness remains controversial. In this study, we created a mouse model in which the IVS2-2A>G mutation was introduced into the mouse Slc26a5 gene by gene targeting. The homozygous Slc26a5 mutant mice were viable and fertile and displayed normal hearing sensitivity by ABR threshold analysis. Whole-mount immunostaining using prestin antibody demonstrated that prestin was correctly targeted to the lateral wall of OHCs, and no obvious hair cell loss occurred in mutant mice. No significant difference in the amount of prestin protein was observed between mutants and controls using western blot analysis. In OHCs isolated from mutants, the NLC was also normal. However, we observed a splicing abnormality in the Slc26a5 mRNA of the mutant mice. Eleven nucleotides were missing from the 5' end of exon 3 in Slc26a5 mRNA, but the normal ATG start codon in exon 3 was still detected. Thus, the IVS2-2A>G mutation in the Slc26a5 gene is insufficient to cause hearing loss in mice. PMID:27232762

  13. Interaction between CFTR and prestin (SLC26A5)

    PubMed Central

    Homma, Kazuaki; Miller, Katharine K.; Anderson, Charles T.; Sengupta, Soma; Du, Guo-Guang; Aguiñaga, Salvador; Cheatham, MaryAnn; Dallos, Peter; Zheng, Jing

    2010-01-01

    Cystic fibrosis transmembrane conductance regulator (CFTR) is a cAMP-activated chloride channel that is present in a variety of epithelial cell types, and usually expressed in the luminal membrane. In contrast, prestin (SLC26A5) is a voltage-dependent motor protein, which is present in the basolateral membrane of cochlear outer hair cells (OHCs), and plays an important role in the frequency selectivity and sensitivity of mammalian hearing. By using in situ hybridization and immunofluorescence, we found that both mRNA and protein of CFTR are present in OHCs, and that CFTR localizes in both the apical and the lateral membranes. CFTR was not detected in the lateral membrane of inner hair cells (IHCs) or in that of OHCs derived from prestin-knockout mice, i.e., in instances where prestin is not expressed. These results suggest that prestin may interact physically with CFTR in the lateral membrane of OHCs. Immunoprecipitation experiments confirmed a prestin-CFTR interaction. Because chloride is important for prestin function and for the efferent-mediated inhibition of cochlear output, the prestin-directed localization of CFTR to the lateral membrane of OHCs has a potential physiological significance. Aside from its role as a chloride channel, CFTR is known as a regulator of multiple protein functions, including those of the solute carrier family 26 (SLC26). Because prestin is in the SLC26 family, several members of which interact with CFTR, we explored the potential modulatory relationship associated with a direct, physical interaction between prestin and CFTR. Electrophysiological experiments demonstrated that cAMP– activated CFTR is capable of enhancing voltage-dependent charge displacement, a signature of OHC motility, whereas prestin does not affect the chloride conductance of CFTR. PMID:20138822

  14. The roles of conserved and nonconserved cysteinyl residues in the oligomerization and function of mammalian prestin.

    PubMed

    Currall, Benjamin; Rossino, Danielle; Jensen-Smith, Heather; Hallworth, Richard

    2011-11-01

    The creation of several prestin knockout and knockin mouse lines has demonstrated the importance of the intrinsic outer hair cell membrane protein prestin to mammalian hearing. However, the structure of prestin remains largely unknown, with even its major features in dispute. Several studies have suggested that prestin forms homo-oligomers that may be stabilized by disulfide bonds. Our phylogenetic analysis of prestin sequences across chordate classes suggested that the cysteinyl residues could be divided into three groups, depending on the extent of their conservation between prestin orthologs and paralogs or homologs. An alanine scan functional analysis was performed of all nine cysteinyl positions in mammalian prestin. Prestin function was assayed by measurement of prestin-associated nonlinear capacitance. Of the nine cysteine-alanine substitution mutations, all were properly membrane targeted and all demonstrated nonlinear capacitance. Four mutations (C124A, C192A, C260A, and C415A), all in nonconserved cysteinyl residues, significantly differed in their nonlinear capacitance properties compared with wild-type prestin. In the two most severely disrupted mutations, substitution of the polar residue seryl for cysteinyl restored normal function in one (C415S) but not the other (C124S). We assessed the relationship of prestin oligomerization to cysteine position using fluorescence resonance energy transfer. With one exception, cysteine-alanine substitutions did not significantly alter prestin-prestin interactions. The exception was C415A, one of the two nonconserved cysteinyl residues whose mutation to alanine caused the most disruption in function. We suggest that no disulfide bond is essential for prestin function. However, C415 likely participates by hydrogen bonding in both nonlinear capacitance and oligomerization. PMID:21813750

  15. Mild Maternal Iron Deficiency Anemia Induces Hearing Impairment Associated with Reduction of Ribbon Synapse Density and Dysregulation of VGLUT3, Myosin VIIa, and Prestin Expression in Young Guinea Pigs.

    PubMed

    Yu, Fei; Hao, Shuai; Yang, Bo; Zhao, Yue; Zhang, Wenyue; Yang, Jun

    2016-05-01

    Mild maternal iron deficiency anemia (IDA) adversely affects the development of cochlear hair cells of the young offspring, but the mechanisms underlying the association are incompletely understood. The aim of this study was to evaluate whether mild maternal IDA in guinea pigs could interrupt inner hair cell (IHC) ribbon synapse density and outer hair cell motility of the offspring. Here, we established a dietary restriction model that allows us to study quantitative changes in the number of IHC ribbon synapses and hearing impairment in response to mild maternal IDA in young guinea pig. The offspring were weaned on postnatal day (PND) 9 and then were given the iron-sufficient diet. On PND 24, pups were examined the hearing function by auditory brainstem response (ABR) and distortion product otoacoustic emission (DPOAE) measurements. Then, the cochleae were harvested for assessment of the number of IHC ribbon synapses by immunofluorescence, the morphology of cochlear hair cells, and spiral ganglion cells (SGCs) by scanning electron microscope and hematoxylin-eosin staining, the location, and expression of vesicular glutamate transporter (VGLUT) 3, myosin VIIa, and prestin by immunofluorescence and blotting. Here, we show that mild maternal IDA in guinea pigs induced elevated ABR threshold shifts, declined DPOAE level shifts, and reduced the number of ribbon synapses, impaired the morphology of cochlear hair cells and SGCs in offsprings. In addition, downregulation of VGLUT3 and myosin VIIa, and upregulation of prestin were observed in the cochlea of offsprings from mild maternal IDA in guinea pigs. These data indicate that mild maternal IDA in guinea pigs induced hearing impairment in offsprings, and this deficit may be attributed to the reduction of ribbon synapse density and dysregulation of VGLUT3, myosin VIIa, and prestin. PMID:26913517

  16. Activity-dependent regulation of prestin expression in mouse outer hair cells

    PubMed Central

    Song, Yohan; Xia, Anping; Lee, Hee Yoon; Wang, Rosalie; Ricci, Anthony J.

    2015-01-01

    Prestin is a membrane protein necessary for outer hair cell (OHC) electromotility and normal hearing. Its regulatory mechanisms are unknown. Several mouse models of hearing loss demonstrate increased prestin, inspiring us to investigate how hearing loss might feedback onto OHCs. To test whether centrally mediated feedback regulates prestin, we developed a novel model of inner hair cell loss. Injection of diphtheria toxin (DT) into adult CBA mice produced significant loss of inner hair cells without affecting OHCs. Thus, DT-injected mice were deaf because they had no afferent auditory input despite OHCs continuing to receive normal auditory mechanical stimulation and having normal function. Patch-clamp experiments demonstrated no change in OHC prestin, indicating that loss of information transfer centrally did not alter prestin expression. To test whether local mechanical feedback regulates prestin, we used TectaC1509G mice, where the tectorial membrane is malformed and only some OHCs are stimulated. OHCs connected to the tectorial membrane had normal prestin levels, whereas OHCs not connected to the tectorial membrane had elevated prestin levels, supporting an activity-dependent model. To test whether the endocochlear potential was necessary for prestin regulation, we studied TectaC1509G mice at different developmental ages. OHCs not connected to the tectorial membrane had lower than normal prestin levels before the onset of the endocochlear potential and higher than normal prestin levels after the onset of the endocochlear potential. Taken together, these data indicate that OHC prestin levels are regulated through local feedback that requires mechanoelectrical transduction currents. This adaptation may serve to compensate for variations in the local mechanical environment. PMID:25810486

  17. Genes and Syndromic Hearing Loss.

    ERIC Educational Resources Information Center

    Keats, Bronya J. B.

    2002-01-01

    This article provides a description of the human genome and patterns of inheritance and discusses genes that are associated with some of the syndromes for which hearing loss is a common finding, including: Waardenburg, Stickler, Jervell and Lange-Neilsen, Usher, Alport, mitochondrial encephalomyopathy, and sensorineural hearing loss. (Contains…

  18. Essential helix interactions in the anion transporter domain of prestin revealed by evolutionary trace analysis.

    PubMed

    Rajagopalan, Lavanya; Patel, Nimish; Madabushi, Srinivasan; Goddard, Julie Anne; Anjan, Venkat; Lin, Feng; Shope, Cindy; Farrell, Brenda; Lichtarge, Olivier; Davidson, Amy L; Brownell, William E; Pereira, Fred A

    2006-12-01

    Prestin, a member of the SLC26A family of anion transporters, is a polytopic membrane protein found in outer hair cells (OHCs) of the mammalian cochlea. Prestin is an essential component of the membrane-based motor that enhances electromotility of OHCs and contributes to frequency sensitivity and selectivity in mammalian hearing. Mammalian cells expressing prestin display a nonlinear capacitance (NLC), widely accepted as the electrical signature of electromotility. The associated charge movement requires intracellular anions reflecting the membership of prestin in the SLC26A family. We used the computational approach of evolutionary trace analysis to identify candidate functional (trace) residues in prestin for mutational studies. We created a panel of mutations at each trace residue and determined membrane expression and nonlinear capacitance associated with each mutant. We observe that several residue substitutions near the conserved sulfate transporter domain of prestin either greatly reduce or eliminate NLC, and the effect is dependent on the size of the substituted residue. These data suggest that packing of helices and interactions between residues surrounding the "sulfate transporter motif" is essential for normal prestin activity. PMID:17151276

  19. A motif of eleven amino acids is a structural adaptation that facilitates motor capability of eutherian prestin

    PubMed Central

    Tan, Xiaodong; Pecka, Jason L.; Tang, Jie; Lovas, Sándor; Beisel, Kirk W.; He, David Z. Z.

    2012-01-01

    Cochlear outer hair cells (OHCs) alter their length in response to transmembrane voltage changes. This so-called electromotility is the result of conformational changes of membrane-bound prestin. Prestin-based OHC motility is thought to be responsible for cochlear amplification, which contributes to the exquisite frequency selectivity and sensitivity of mammalian hearing. Prestin belongs to an anion transporter family, the solute carrier protein 26A (SLC26A). Prestin is unique in this family in that it functions as a voltage-dependent motor protein manifested by two hallmarks, nonlinear capacitance and motility. Evidence suggests that prestin orthologs from zebrafish and chicken are anion exchangers or transporters with no motor function. We identified a segment of 11 amino acid residues in eutherian prestin that is extremely conserved among eutherian species but highly variable among non-mammalian orthologs and SLC26A paralogs. To determine whether this sequence represents a motif that facilitates motor function in eutherian prestin, we utilized a chimeric approach by swapping corresponding residues from the zebrafish and chicken with those of gerbil. Motility and nonlinear capacitance were measured from chimeric prestin-transfected human embryonic kidney 293 cells using a voltage-clamp technique and photodiode-based displacement measurement system. We observed a gain of motor function with both of the hallmarks in the chimeric prestin without loss of transport function. Our results show, for the first time, that the substitution of a span of 11 amino acid residues confers the electrogenic anion transporters of zebrafish and chicken prestins with motor-like function. Thus, this motif represents the structural adaptation that assists gain of motor function in eutherian prestin. PMID:22399806

  20. A motif of eleven amino acids is a structural adaptation that facilitates motor capability of eutherian prestin.

    PubMed

    Tan, Xiaodong; Pecka, Jason L; Tang, Jie; Lovas, Sándor; Beisel, Kirk W; He, David Z Z

    2012-02-15

    Cochlear outer hair cells (OHCs) alter their length in response to transmembrane voltage changes. This so-called electromotility is the result of conformational changes of membrane-bound prestin. Prestin-based OHC motility is thought to be responsible for cochlear amplification, which contributes to the exquisite frequency selectivity and sensitivity of mammalian hearing. Prestin belongs to an anion transporter family, the solute carrier protein 26A (SLC26A). Prestin is unique in this family in that it functions as a voltage-dependent motor protein manifested by two hallmarks, nonlinear capacitance and motility. Evidence suggests that prestin orthologs from zebrafish and chicken are anion exchangers or transporters with no motor function. We identified a segment of 11 amino acid residues in eutherian prestin that is extremely conserved among eutherian species but highly variable among non-mammalian orthologs and SLC26A paralogs. To determine whether this sequence represents a motif that facilitates motor function in eutherian prestin, we utilized a chimeric approach by swapping corresponding residues from the zebrafish and chicken with those of gerbil. Motility and nonlinear capacitance were measured from chimeric prestin-transfected human embryonic kidney 293 cells using a voltage-clamp technique and photodiode-based displacement measurement system. We observed a gain of motor function with both of the hallmarks in the chimeric prestin without loss of transport function. Our results show, for the first time, that the substitution of a span of 11 amino acid residues confers the electrogenic anion transporters of zebrafish and chicken prestins with motor-like function. Thus, this motif represents the structural adaptation that assists gain of motor function in eutherian prestin. PMID:22399806

  1. Evaluating Prestin's Changing Biophysical Attributes in Development Using a Tet-Induced Cell Line

    NASA Astrophysics Data System (ADS)

    Bian, Shumin; Koo, Bon W.; Kelleher, Stephen; Santos-Sacchi, Joseph; Navaratnam, Dhasakumar

    2011-11-01

    The motor protein prestin is a member of the SLC26 anion transporter family, and expressed in the lateral wall of OHCs. It is now widely recognized that prestin is required for mammalian cochlear amplification. Expression of prestin precedes the onset of hearing in mice and undergoes a functional maturation within the membrane coincident with the onset of hearing. We have developed several tetracycline-inducible prestin expressing cell lines that duplicate prestins functional maturation in vivo. Thus, following induction there is an initial stage of increase in the charge carried by an individual motor (z) that accompanies a phase of slow growth in charge density. A plateau in z follows and is accompanied by rapid increase in charge density. The latter strongly correlates with an increasing ratio between an apparently larger and smaller monomer of prestin, suggesting that the latter exerts a dominant negative effect on function. Through the experimental period there is a progressive shift in the voltage of peak capacitance, similar to that observed in developing OHCs. Finally, we observed a non-selective ionic current after induction, the size of which correlated with charge density.

  2. Parallel signatures of sequence evolution among hearing genes in echolocating mammals: an emerging model of genetic convergence.

    PubMed

    Davies, K T J; Cotton, J A; Kirwan, J D; Teeling, E C; Rossiter, S J

    2012-05-01

    Recent findings of sequence convergence in the Prestin gene among some bats and cetaceans suggest that parallel adaptations for high-frequency hearing have taken place during the evolution of echolocation. To determine if this gene is an exception, or instead similar processes have occurred in other hearing genes, we have examined Tmc1 and Pjvk, both of which are associated with non-syndromic hearing loss in mammals. These genes were amplified and sequenced from a number of mammalian species, including echolocating and non-echolocating bats and whales, and were analysed together with published sequences. Sections of both genes showed phylogenetic signals that conflicted with accepted species relationships, with coding regions uniting laryngeal echolocating bats in a monophyletic clade. Bayesian estimates of posterior probabilities of convergent and divergent substitutions provided more direct evidence of sequence convergence between the two groups of laryngeal echolocating bats as well as between echolocating bats and dolphins. We found strong evidence of positive selection acting on some echolocating bat species and echolocating cetaceans, contrasting with purifying selection on non-echolocating bats. Signatures of sequence convergence and molecular adaptation in two additional hearing genes suggest that the acquisition of high-frequency hearing has involved multiple loci. PMID:22167055

  3. Prestin at Year 14: Progress and Prospect

    PubMed Central

    He, David Z.Z.; Lovas, Sándor; Ai, Yu; Li, Yi; Beisel, Kirk W.

    2014-01-01

    Prestin, the motor protein of cochlear outer hair cells, was identified 14 years ago. Prestin-based outer hair cell motility is responsible for the exquisite sensitivity and frequency selectivity seen in the mammalian cochlea. Prestin is the 5th member of an eleven-member membrane transporter superfamily of SLC26A proteins. Unlike its paralogs, which are capable of transporting anions across the cell membrane, prestin primarily functions as a motor protein with unique capability of performing direct and reciprocal electromechanical conversion on microsecond time scale. Significant progress in the understanding of its structure and the molecular mechanism has been made in recent years using electrophysiological, biochemical, comparative genomics, structural bioinformatics, molecular dynamics simulation, site-directed mutagenesis and domain-swapping techniques. This article reviews recent advances of the structural and functional properties of prestin with focus on the areas that are critical but still controversial in understanding the molecular mechanism of how prestin works: The structural domains for voltage sensing and interaction with anions and for conformational change. Future research directions and potential application of prestin are also discussed. PMID:24361298

  4. The Evolution of Prestin: Examination with Membrane Thickness Sensitivity

    NASA Astrophysics Data System (ADS)

    Izumi, Chisako; Bird, Jonathan; Schächinger, Torsten; Oliver, Dominik; Iwasa, Kuni H.

    2011-11-01

    Prestin (SLC26A5) is a membrane protein that is essential for electromotility of outer hair cells and, in turn, for the cochlear amplifier. This function is mammalian innovation and prestin orthologs in non-mammalian hair cells function as anion exchangers rather than a motile element. Taking advantage of this evolutionary change, we found that the sensitivity of prestin orthologs to membrane thickness is inversely related to their effectiveness for the motile function. In addition, a chimera between zebrafish prestin and rat prestin, which shows motile functionality, has membrane thickness sensitivity lower than any prestin ortholog we have examined, confirming the relationship.

  5. Lizard and Frog Prestin: Evolutionary Insight into Functional Changes

    PubMed Central

    Tang, Jie; Pecka, Jason L.; Fritzsch, Bernd

    2013-01-01

    The plasma membrane of mammalian cochlear outer hair cells contains prestin, a unique motor protein. Prestin is the fifth member of the solute carrier protein 26A family. Orthologs of prestin are also found in the ear of non-mammalian vertebrates such as zebrafish and chicken. However, these orthologs are electrogenic anion exchangers/transporters with no motor function. Amphibian and reptilian lineages represent phylogenic branches in the evolution of tetrapods and subsequent amniotes. Comparison of the peptide sequences and functional properties of these prestin orthologs offer new insights into prestin evolution. With the recent availability of the lizard and frog genome sequences, we examined amino acid sequence and function of lizard and frog prestins to determine how they are functionally and structurally different from prestins of mammals and other non-mammals. Somatic motility, voltage-dependent nonlinear capacitance (NLC), the two hallmarks of prestin function, and transport capability were measured in transfected human embryonic kidney cells using voltage-clamp and radioisotope techniques. We demonstrated that while the transport capability of lizard and frog prestin was compatible to that of chicken prestin, the NLC of lizard prestin was more robust than that of chicken’s and was close to that of platypus. However, unlike platypus prestin which has acquired motor capability, lizard or frog prestin did not demonstrate motor capability. Lizard and frog prestins do not possess the same 11-amino-acid motif that is likely the structural adaptation for motor function in mammals. Thus, lizard and frog prestins appear to be functionally more advanced than that of chicken prestin, although motor capability is not yet acquired. PMID:23342145

  6. Lizard and frog prestin: evolutionary insight into functional changes.

    PubMed

    Tang, Jie; Pecka, Jason L; Fritzsch, Bernd; Beisel, Kirk W; He, David Z Z

    2013-01-01

    The plasma membrane of mammalian cochlear outer hair cells contains prestin, a unique motor protein. Prestin is the fifth member of the solute carrier protein 26A family. Orthologs of prestin are also found in the ear of non-mammalian vertebrates such as zebrafish and chicken. However, these orthologs are electrogenic anion exchangers/transporters with no motor function. Amphibian and reptilian lineages represent phylogenic branches in the evolution of tetrapods and subsequent amniotes. Comparison of the peptide sequences and functional properties of these prestin orthologs offer new insights into prestin evolution. With the recent availability of the lizard and frog genome sequences, we examined amino acid sequence and function of lizard and frog prestins to determine how they are functionally and structurally different from prestins of mammals and other non-mammals. Somatic motility, voltage-dependent nonlinear capacitance (NLC), the two hallmarks of prestin function, and transport capability were measured in transfected human embryonic kidney cells using voltage-clamp and radioisotope techniques. We demonstrated that while the transport capability of lizard and frog prestin was compatible to that of chicken prestin, the NLC of lizard prestin was more robust than that of chicken's and was close to that of platypus. However, unlike platypus prestin which has acquired motor capability, lizard or frog prestin did not demonstrate motor capability. Lizard and frog prestins do not possess the same 11-amino-acid motif that is likely the structural adaptation for motor function in mammals. Thus, lizard and frog prestins appear to be functionally more advanced than that of chicken prestin, although motor capability is not yet acquired. PMID:23342145

  7. Basilar Membrane Measurements from Wild Type, Prestin 499, and Prestin KO mice

    NASA Astrophysics Data System (ADS)

    Weddell, Thomas; Mellado-Lagarde, Marcia; Lukashkina, Victoria; Lukashkin, Andrei; Zuo, Jian; Russell, Ian

    2011-11-01

    It has been predicted that a nonfunctional prestin in the mammalian cochlea would produce a basilar membrane response at lower characteristic frequency, as we see in the prestin knock-out mouse, but with a reduced sensitivity that would reflect an enhanced coupling between basilar membrane and reticular lamina and inner hair cell stereocilia. We demonstrate here that this is the case in measurements from the 499 mouse where prestin in the lateral membrane of the outer hair cells is present but effectively silenced.

  8. Cochlear amplification, outer hair cells and prestin

    PubMed Central

    Dallos, Peter

    2008-01-01

    Mechanical amplification of acoustic signals is apparently a common feature of vertebrate auditory organs. In non-mammalian vertebrates amplification is produced by stereociliary processes, related to the mechanotransducer channel complex and probably to the phenomenon of fast adaptation. The extended frequency range of the mammalian cochlea has likely co-evolved with a novel hair cell type, the outer hair cell and its constituent membrane protein, prestin. Cylindrical outer hair cells are motile and their somatic length changes are voltage driven and powered by prestin. One of the central outstanding problems in mammalian cochlear neurobiology is the relation between the two amplification processes. PMID:18809494

  9. Prestin's anion transport and voltage-sensing capabilities are independent.

    PubMed

    Bai, Jun-Ping; Surguchev, Alexei; Montoya, Simone; Aronson, Peter S; Santos-Sacchi, Joseph; Navaratnam, Dhasakumar

    2009-04-22

    The integral membrane protein prestin, a member of the SLC26 anion transporter family, is responsible for the voltage-driven electromotility of mammalian outer hair cells. It was argued that the evolution of prestin's motor function required a loss of the protein's transport capabilities. Instead, it was proposed that prestin manages only an abortive hemicycle that results in the trapped anion acting as a voltage sensor, to generate the motor's signature gating charge movement or nonlinear capacitance. We demonstrate, using classical radioactive anion ([(14)C]formate and [(14)C]oxalate) uptake studies, that in contrast to previous observations, prestin is able to transport anions. The prestin-dependent uptake of both these anions was twofold that of cells transfected with vector alone, and comparable to SLC26a6, prestin's closest phylogenetic relative. Furthermore, we identify a potential chloride-binding site in which the mutations of two residues (P328A and L326A) preserve nonlinear capacitance, yet negate anion transport. Finally, we distinguish 12 charged residues out of 22, residing within prestin's transmembrane regions, that contribute to unitary charge movement, i.e., voltage sensing. These data redefine our mechanistic concept of prestin. PMID:19383462

  10. Prestin forms oligomer with four mechanically independent subunits

    PubMed Central

    Wang, Xiang; Yang, Shiming; Jia, Shuping; He, David Z.Z.

    2010-01-01

    Prestin is the motor protein of cochlear outer hair cells (OHCs) with the unique capability of performing direct, rapid and reciprocal electromechanical conversion. Prestin consists of 744 amino acids with a molecular mass of ~81.4 kDa. The predicted membrane topology and molecular mass of a single prestin molecule appear inadequate to account for the size of intramembrane particles (IMPs) expressed in the OHC membrane. Although recent biochemical evidence suggests that prestin forms homo-oligomers, most likely as a tetramer, the oligomeric structure of prestin in OHCs remains unclear. We obtained the charge density of prestin in the gerbil OHCs by measuring their nonlinear capacitance (NLC). The average charge density (22,608 μm−2) measured was four times the average IMP density (5,686 μm−2) reported in the freeze-fracture study. This suggests that each IMP contains four prestin molecules, based on the general notion that each prestin transfers a single elementary charge. We subsequently compared the voltage dependency and the values of slope factor of NLC and somatic motility simultaneously measured from the same OHCs to determine whether NLC and motility are fully coupled and how prestin subunits function within the tetramer. We showed that the voltage dependency and slope factors of NLC and motility were not statistically different, suggesting that NLC and motility are fully coupled. The fact that the slope factor is the same between NLC and motility suggests that each prestin monomer in the tetramer is in parallel, each interacting independently with cytoplasmic or other partners to facilitate the mechanical response. PMID:20347723

  11. Cloning genes for non-syndromal hearing impairment.

    PubMed

    Smith, R J; Van Camp, G

    1999-10-01

    Over 45 genes that cause autosomal non-syndromic hearing impairment (NSHI) have been localized and many more are predicted to exist. To clone these genes, a number of different strategies can be used. This paper focuses on four general approaches: functional cloning, positional cloning, position-dependent candidate gene cloning, and position-independent candidate gene cloning. PMID:10890140

  12. Tyrosine motifs are required for prestin basolateral membrane targeting

    PubMed Central

    Zhang, Yifan; Moeini-Naghani, Iman; Bai, JunPing; Santos-Sacchi, Joseph; Navaratnam, Dhasakumar S.

    2015-01-01

    ABSTRACT Prestin is targeted to the lateral wall of outer hair cells (OHCs) where its electromotility is critical for cochlear amplification. Using MDCK cells as a model system for polarized epithelial sorting, we demonstrate that prestin uses tyrosine residues, in a YXXΦ motif, to target the basolateral surface. Both Y520 and Y667 are important for basolateral targeting of prestin. Mutation of these residues to glutamine or alanine resulted in retention within the Golgi and delayed egress from the Golgi in Y667Q. Basolateral targeting is restored upon mutation to phenylalanine suggesting the importance of a phenol ring in the tyrosine side chain. We also demonstrate that prestin targeting to the basolateral surface is dependent on AP1B (μ1B), and that prestin uses transferrin containing early endosomes in its passage from the Golgi to the basolateral plasma membrane. The presence of AP1B (μ1B) in OHCs, and parallels between prestin targeting to the basolateral surface of OHCs and polarized epithelial cells suggest that outer hair cells resemble polarized epithelia rather than neurons in this important phenotypic measure. PMID:25596279

  13. Treating hearing disorders with cell and gene therapy

    NASA Astrophysics Data System (ADS)

    Gillespie, Lisa N.; Richardson, Rachael T.; Nayagam, Bryony A.; Wise, Andrew K.

    2014-12-01

    Hearing loss is an increasing problem for a substantial number of people and, with an aging population, the incidence and severity of hearing loss will become more significant over time. There are very few therapies currently available to treat hearing loss, and so the development of new therapeutic strategies for hearing impaired individuals is of paramount importance to address this unmet clinical need. Most forms of hearing loss are progressive in nature and therefore an opportunity exists to develop novel therapeutic approaches to slow or halt hearing loss progression, or even repair or replace lost hearing function. Numerous emerging technologies have potential as therapeutic options. This paper details the potential of cell- and gene-based therapies to provide therapeutic agents to protect sensory and neural cells from various insults known to cause hearing loss; explores the potential of replacing lost sensory and nerve cells using gene and stem cell therapy; and describes the considerations for clinical translation and the challenges that need to be overcome.

  14. Membrane thickness sensitivity of prestin orthologs: the evolution of a piezoelectric protein.

    PubMed

    Izumi, Chisako; Bird, Jonathan E; Iwasa, Kuni H

    2011-06-01

    How proteins evolve new functionality is an important question in biology; prestin (SLC26A5) is a case in point. Prestin drives outer hair cell somatic motility and amplifies mechanical vibrations in the mammalian cochlea. The motility of mammalian prestin is analogous to piezoelectricity, in which charge transfer is coupled to changes in membrane area occupied by the protein. Intriguingly, nonmammalian prestin orthologs function as anion exchangers but are apparently nonmotile. We previously found that mammalian prestin is sensitive to membrane thickness, suggesting that prestin's extended conformation has a thinner hydrophobic height in the lipid bilayer. Because prestin-based motility is a mammalian specialization, we initially hypothesized that nonmotile prestin orthologs, while functioning as anion transporters, should be much less sensitive to membrane thickness. We found the exact opposite to be true. Chicken prestin was the most sensitive to thickness changes, displaying the largest shift in voltage dependence. Platypus prestin displayed an intermediate response to membrane thickness and gerbil prestin was the least sensitive. To explain these observations, we present a theory where force production, rather than displacement, was selected for the evolution of prestin as a piezoelectric membrane motor. PMID:21641306

  15. The V499G/Y501H Mutation Impairs Fast Motor Kinetics of Prestin and Has Significance for Defining Functional Independence of Individual Prestin Subunits*

    PubMed Central

    Homma, Kazuaki; Duan, Chongwen; Zheng, Jing; Cheatham, Mary Ann; Dallos, Peter

    2013-01-01

    Outer hair cells (OHCs) are a mammalian innovation for mechanically amplifying sound energy to overcome the viscous damping of the cochlear partition. Although the voltage-dependent OHC membrane motor, prestin, has been demonstrated to be essential for mammalian cochlear amplification, the molecular mechanism by which prestin converts electrical energy into mechanical displacement/force remains elusive. Identifying mutations that alter the motor function of prestin provides vital information for unraveling the energy transduction mechanism of prestin. We show that the V499G/Y501H mutation does not deprive prestin of its voltage-induced motor activity, but it does significantly impair the fast motor kinetics and voltage operating range. Furthermore, mutagenesis studies suggest that Val-499 is the primary site responsible for these changes. We also show that V499G/Y501H prestin forms heteromers with wild-type prestin and that the fast motor kinetics of wild-type prestin is not affected by heteromer formation with V499G/Y501H prestin. These results suggest that prestin subunits are individually functional within a given multimer. PMID:23212912

  16. HEI-OC1 cells as a model for investigating prestin function.

    PubMed

    Park, Channy; Thein, Pru; Kalinec, Gilda; Kalinec, Federico

    2016-05-01

    The House Ear Institute-Organ of Corti 1 (HEI-OC1) is a mouse auditory cell line that endogenously express, among other several markers of cochlear hair cells, the motor protein prestin (SLC26A5). Since its discovery fifteen years ago, and because of the difficulties associated with working with outer hair cells, prestin studies have been performed mostly by expressing it exogenously in non-specific systems such as HEK293 and TSA201, embryonic kidney cells from human origin, or Chinese Hamster Ovary (CHO) cells. Here, we report flow cytometry and confocal laser scanning microscopy studies on the pattern of prestin expression, as well as nonlinear capacitance (NLC) and whole cell-patch clamping studies on prestin motor function, in HEI-OC1 cells cultured at permissive and non-permissive conditions. Our results indicate that both total prestin expression and plasma membrane localization increase in a time-dependent manner when HEI-OC1 cells differentiate under non-permissive culture conditions. In addition, we demonstrate that HEI-OC1 cells have a robust NLC associated to prestin motor function, which decreases when the density of prestin molecules present at the plasma membrane increases. Altogether, our results show that the response of endogenously expressed prestin in HEI-OC1 cells is different from the response of prestin expressed exogenously in non-auditory cells, and suggest that the HEI-OC1 cell line may be an important additional tool for investigating prestin function. PMID:26854618

  17. Selective cell-surface labeling of the molecular motor protein prestin

    SciTech Connect

    McGuire, Ryan M.; Silberg, Jonathan J.; Pereira, Fred A.; Raphael, Robert M.

    2011-06-24

    Highlights: {yields} Trafficking to the plasma membrane is required for prestin function. {yields} Biotin acceptor peptide (BAP) was fused to prestin through a transmembrane domain. {yields} BAP-prestin can be metabolically labeled with biotin in HEK293 cells. {yields} Biotin-BAP-prestin allows for selective imaging of fully trafficked prestin. {yields} The biotin-BAP-prestin displays voltage-sensitive activity. -- Abstract: Prestin, a multipass transmembrane protein whose N- and C-termini are localized to the cytoplasm, must be trafficked to the plasma membrane to fulfill its cellular function as a molecular motor. One challenge in studying prestin sequence-function relationships within living cells is separating the effects of amino acid substitutions on prestin trafficking, plasma membrane localization and function. To develop an approach for directly assessing prestin levels at the plasma membrane, we have investigated whether fusion of prestin to a single pass transmembrane protein results in a functional fusion protein with a surface-exposed N-terminal tag that can be detected in living cells. We find that fusion of the biotin-acceptor peptide (BAP) and transmembrane domain of the platelet-derived growth factor receptor (PDGFR) to the N-terminus of prestin-GFP yields a membrane protein that can be metabolically-labeled with biotin, trafficked to the plasma membrane, and selectively detected at the plasma membrane using fluorescently-tagged streptavidin. Furthermore, we show that the addition of a surface detectable tag and a single-pass transmembrane domain to prestin does not disrupt its voltage-sensitive activity.

  18. From Zebrafish to Mammal: Functional Evolution of Prestin, the Motor Protein of Cochlear Outer Hair Cells

    PubMed Central

    Tan, Xiaodong; Pecka, Jason L.; Tang, Jie; Okoruwa, Oseremen E.; Zhang, Qian

    2011-01-01

    Prestin is the motor protein of cochlear outer hair cells. It belongs to a distinct anion transporter family called solute carrier protein 26A, or SLC26A. Members of this family serve two fundamentally distinct functions. Although most members transport different anion substrates across a variety of epithelia, prestin (SLC26A5) is unique, functioning as a voltage-dependent motor protein. Recent evidence suggests that prestin orthologs from zebrafish and chicken are electrogenic divalent/chloride anion exchangers/transporters with no motor function. These studies appear to suggest that prestin was evolved from an anion transporter. We examined the motor and transport functions of prestin and its orthologs from four different species in the vertebrate lineage, to gain insights of how these two physiological functions became distinct. Somatic motility, voltage-dependent nonlinear capacitance (NLC), and transporter function were measured in transfected human embryonic kidney (HEK) cells using voltage-clamp and anion uptake techniques. Zebrafish and chicken prestins both exhibited weak NLC, with peaks significantly shifted in the depolarization (right) direction. This was contrasted by robust NLC with peaks left shifted in the platypus and gerbil. The platypus and gerbil prestins retained little transporter function compared with robust anion transport capacities in the zebrafish and chicken orthologs. Somatic motility was detected only in the platypus and gerbil prestins. There appears to be an inverse relationship between NLC and anion transport functions, whereas motor function appears to have emerged only in mammalian prestin. Our results suggest that motor function is an innovation of therian prestin and is concurrent with diminished transporter capabilities. PMID:21047933

  19. From zebrafish to mammal: functional evolution of prestin, the motor protein of cochlear outer hair cells.

    PubMed

    Tan, Xiaodong; Pecka, Jason L; Tang, Jie; Okoruwa, Oseremen E; Zhang, Qian; Beisel, Kirk W; He, David Z Z

    2011-01-01

    Prestin is the motor protein of cochlear outer hair cells. It belongs to a distinct anion transporter family called solute carrier protein 26A, or SLC26A. Members of this family serve two fundamentally distinct functions. Although most members transport different anion substrates across a variety of epithelia, prestin (SLC26A5) is unique, functioning as a voltage-dependent motor protein. Recent evidence suggests that prestin orthologs from zebrafish and chicken are electrogenic divalent/chloride anion exchangers/transporters with no motor function. These studies appear to suggest that prestin was evolved from an anion transporter. We examined the motor and transport functions of prestin and its orthologs from four different species in the vertebrate lineage, to gain insights of how these two physiological functions became distinct. Somatic motility, voltage-dependent nonlinear capacitance (NLC), and transporter function were measured in transfected human embryonic kidney (HEK) cells using voltage-clamp and anion uptake techniques. Zebrafish and chicken prestins both exhibited weak NLC, with peaks significantly shifted in the depolarization (right) direction. This was contrasted by robust NLC with peaks left shifted in the platypus and gerbil. The platypus and gerbil prestins retained little transporter function compared with robust anion transport capacities in the zebrafish and chicken orthologs. Somatic motility was detected only in the platypus and gerbil prestins. There appears to be an inverse relationship between NLC and anion transport functions, whereas motor function appears to have emerged only in mammalian prestin. Our results suggest that motor function is an innovation of therian prestin and is concurrent with diminished transporter capabilities. PMID:21047933

  20. Selective cell-surface labeling of the molecular motor protein prestin

    PubMed Central

    McGuire, Ryan M.; Silberg, Jonathan J.; Pereira, Fred A.; Raphael, Robert M.

    2011-01-01

    Prestin, a multipass transmembrane protein whose N- an C-termini are localized to the cytoplasm, must be trafficked to the plasma membrane to fulfill its cellular function as a molecular motor. One challenge in studying prestin sequence-function relationships within living cells is separating the effects of amino acid substitutions on prestin trafficking, plasma membrane localization and function. To develop an approach for directly assessing prestin levels at the plasma membrane, we have investigated whether fusion of prestin to a single pass transmembrane protein results in a functional fusion protein with a surface-exposed N-terminal tag that can be detected in living cells. We find that fusion of the biotin-acceptor peptide (BAP) and transmembrane domain of the platelet-derived growth factor receptor (PDGFR) to the N-terminus of prestin-GFP yields a membrane protein that can be metabolically-labeled with biotin, trafficked to the plasma membrane, and selectively detected at the plasma membrane using fluorescently-tagged streptavidin. Furthermore, we show that the addition of a surface detectable tag and a single-pass transmembrane domain to prestin does not disrupt its voltage-sensitive activity. PMID:21651892

  1. Selective cell-surface labeling of the molecular motor protein prestin.

    PubMed

    McGuire, Ryan M; Silberg, Jonathan J; Pereira, Fred A; Raphael, Robert M

    2011-06-24

    Prestin, a multipass transmembrane protein whose N- and C-termini are localized to the cytoplasm, must be trafficked to the plasma membrane to fulfill its cellular function as a molecular motor. One challenge in studying prestin sequence-function relationships within living cells is separating the effects of amino acid substitutions on prestin trafficking, plasma membrane localization and function. To develop an approach for directly assessing prestin levels at the plasma membrane, we have investigated whether fusion of prestin to a single pass transmembrane protein results in a functional fusion protein with a surface-exposed N-terminal tag that can be detected in living cells. We find that fusion of the biotin-acceptor peptide (BAP) and transmembrane domain of the platelet-derived growth factor receptor (PDGFR) to the N-terminus of prestin-GFP yields a membrane protein that can be metabolically-labeled with biotin, trafficked to the plasma membrane, and selectively detected at the plasma membrane using fluorescently-tagged streptavidin. Furthermore, we show that the addition of a surface detectable tag and a single-pass transmembrane domain to prestin does not disrupt its voltage-sensitive activity. PMID:21651892

  2. Combination of hearing screening and genetic screening for deafness-susceptibility genes in newborns

    PubMed Central

    YAO, GEN-DONG; LI, SHOU-XIA; CHEN, DING-LI; FENG, HAI-QIN; ZHAO, SU-BIN; LIU, YONG-JIE; GUO, LI-LI; YANG, ZHI-MING; ZHANG, XIAO-FANG; SUN, CAI-XIA; WANG, ZE-HUI; ZHANG, WEI-YONG

    2014-01-01

    The aim of this study was to determine the clinical significance of the results of screening of newborn hearing and the incidence of deafness-susceptibility genes. One thousand newborn babies in the Handan Center Hospital (Handan, China) underwent screening of hearing and deafness-susceptibility genes. The first screening test was carried out using otoacoustic emissions (OAEs). Babies with hearing loss who failed to pass the initial screening were scheduled for rescreening at 42 days after birth. Cord blood was used for the screening of deafness-susceptibility genes, namely the GJB2, SLC26A4 and mitochondrial 12S rRNA (MTRNR1) genes. Among the 1,000 neonates that underwent the first hearing screening, 25 exhibited left-sided hearing loss, 21 exhibited right-sided hearing loss and 15 cases had binaural hearing loss. After rescreening 42 days later, only one of the initial 61 cases exhibited hearing loss under OAE testing. The neonatal deafness gene tests showed two cases with 1555A>G mutation and two cases with 1494C>T mutation of the MTRNR1 gene. In the SLC26A4 gene screening, four cases exhibited the heterozygous IVS7-2A>G mutation and one case exhibited heterozygous 1226G>A mutation. In the GJB2 gene screening, two cases exhibited the homozygous 427C>T mutation and 10 exhibited the heterozygous 235delC mutation. The genetic screening revealed 21 newborns with mutations in the three deafness-susceptibility genes. The overall carrier rate was 2.1% (21/1,000). The association of hearing and gene screening may be the promising screening strategy for the diagnosis of hearing loss. PMID:24348793

  3. Hearing

    ERIC Educational Resources Information Center

    Koehlinger, Keegan M.; Van Horne, Amanda J. Owen; Moeller, Mary Pat

    2013-01-01

    Purpose: Spoken language skills of 3- and 6-year-old children who are hard of hearing (HH) were compared with those of children with normal hearing (NH). Method: Language skills were measured via mean length of utterance in words (MLUw) and percent correct use of finite verb morphology in obligatory contexts based on spontaneous conversational…

  4. Sensitivity of prestin-based membrane motor to membrane thickness.

    PubMed

    Fang, Jie; Izumi, Chisako; Iwasa, Kuni H

    2010-06-16

    Prestin is the membrane protein in outer hair cells that harnesses electrical energy by changing its membrane area in response to changes in the membrane potential. To examine the effect of membrane thickness on this protein, phosphatidylcholine (PC) with various acyl-chain lengths were incorporated into the plasma membrane by using gamma-cyclodextrin. Incorporation of short chain PCs increased the linear capacitance and positively shifted the voltage dependence of prestin, up to 120 mV, in cultured cells. PCs with long acyl chains had the opposite effects. Because the linear capacitance is inversely related to the membrane thickness, these voltage shifts are attributable to membrane thickness. The corresponding voltage shifts of electromotility were observed in outer hair cells. These results demonstrate that electromotility is extremely sensitive to the thickness of the plasma membrane, presumably involving hydrophobic mismatch. These observations indicate that the extended state of the motor molecule, which is associated with the elongation of outer hair cells, has a conformation with a shorter hydrophobic height in the lipid bilayer. PMID:20550895

  5. Intracellular calcium affects prestin's voltage operating point indirectly via turgor-induced membrane tension

    NASA Astrophysics Data System (ADS)

    Song, Lei; Santos-Sacchi, Joseph

    2015-12-01

    Recent identification of a calmodulin binding site within prestin's C-terminus indicates that calcium can significantly alter prestin's operating voltage range as gauged by the Boltzmann parameter Vh (Keller et al., J. Neuroscience, 2014). We reasoned that those experiments may have identified the molecular substrate for the protein's tension sensitivity. In an effort to understand how this may happen, we evaluated the effects of turgor pressure on such shifts produced by calcium. We find that the shifts are induced by calcium's ability to reduce turgor pressure during whole cell voltage clamp recording. Clamping turgor pressure to 1kPa, the cell's normal intracellular pressure, completely counters the calcium effect. Furthermore, following unrestrained shifts, collapsing the cells abolishes induced shifts. We conclude that calcium does not work by direct action on prestin's conformational state. The possibility remains that calcium interaction with prestin alters water movements within the cell, possibly via its anion transport function.

  6. Phosphodiesterase 4D gene polymorphisms in sudden sensorineural hearing loss.

    PubMed

    Chien, Chen-Yu; Tai, Shu-Yu; Wang, Ling-Feng; Hsi, Edward; Chang, Ning-Chia; Wang, Hsun-Mo; Wu, Ming-Tsang; Ho, Kuen-Yao

    2016-09-01

    The phosphodiesterase 4D (PDE4D) gene has been reported as a risk gene for ischemic stroke. The vascular factors are between the hypothesized etiologies of sudden sensorineural hearing loss (SSNHL), and this genetic effect might be attributed for its role in SSNHL. We hypothesized that genetic variants of the PDE4D gene are associated with susceptibility to SSNHL. We conducted a case-control study with 362 SSNHL cases and 209 controls. Three single nucleotide polymorphisms (SNPs) were selected. The genotypes were determined using TaqMan technology. Hardy-Weinberg equilibrium (HWE) was tested for each SNP, and genetic effects were evaluated according to three inheritance modes. We carried out sex-specific analysis to analyze the overall data. All three SNPs were in HWE. When subjects were stratified by sex, the genetic effect was only evident in females but not in males. The TT genotype of rs702553 exhibited an adjusted odds ratio (OR) of 3.83 (95 % confidence interval = 1.46-11.18) (p = 0.006) in female SSNHL. The TT genotype of SNP rs702553 was associated with female SSNHL under the recessive model (p = 0.004, OR 3.70). In multivariate logistic regression analysis, TT genotype of rs702553 was significantly associated with female SSNHL (p = 0.0043, OR 3.70). These results suggest that PDE4D gene polymorphisms influence the susceptibility for the development of SSNHL in the southern Taiwanese female population. PMID:26521189

  7. The STAS domain of mammalian SLC26A5 prestin harbours an anion-binding site.

    PubMed

    Lolli, Graziano; Pasqualetto, Elisa; Costanzi, Elisa; Bonetto, Greta; Battistutta, Roberto

    2016-02-15

    Prestin is a unique ATP- and Ca(2+)-independent molecular motor with piezoelectric characteristics responsible for the electromotile properties of mammalian cochlear outer hair cells, i.e. the capacity of these cells to modify their length in response to electric stimuli. This 'electromotility' is at the basis of the exceptional sensitivity and frequency selectivity distinctive of mammals. Prestin belongs to the SLC26 (solute carrier 26) family of anion transporters and needs anions to function properly, particularly Cl(-). In the present study, using X-ray crystallography we reveal that the STAS (sulfate transporter and anti-sigma factor antagonist) domain of mammalian prestin, considered an 'incomplete' transporter, harbours an unanticipated anion-binding site. In parallel, we present the first crystal structure of a prestin STAS domain from a non-mammalian vertebrate prestin (chicken) that behaves as a 'full' transporter. Notably, in chicken STAS, the anion-binding site is lacking because of a local structural rearrangement, indicating that the presence of the STAS anion-binding site is exclusive to mammalian prestin. PMID:26635354

  8. Targeted next-generation sequencing of deafness genes in hearing-impaired individuals uncovers informative mutations

    PubMed Central

    Vona, Barbara; Müller, Tobias; Nanda, Indrajit; Neuner, Cordula; Hofrichter, Michaela A. H.; Schröder, Jörg; Bartsch, Oliver; Läßig, Anne; Keilmann, Annerose; Schraven, Sebastian; Kraus, Fabian; Shehata-Dieler, Wafaa; Haaf, Thomas

    2014-01-01

    Purpose: Targeted next-generation sequencing provides a remarkable opportunity to identify variants in known disease genes, particularly in extremely heterogeneous disorders such as nonsyndromic hearing loss. The present study attempts to shed light on the complexity of hearing impairment. Methods: Using one of two next-generation sequencing panels containing either 80 or 129 deafness genes, we screened 30 individuals with nonsyndromic hearing loss (from 23 unrelated families) and analyzed 9 normal-hearing controls. Results: Overall, we found an average of 3.7 variants (in 80 genes) with deleterious prediction outcome, including a number of novel variants, in individuals with nonsyndromic hearing loss and 1.4 in controls. By next-generation sequencing alone, 12 of 23 (52%) probands were diagnosed with monogenic forms of nonsyndromic hearing loss; one individual displayed a DNA sequence mutation together with a microdeletion. Two (9%) probands have Usher syndrome. In the undiagnosed individuals (10/23; 43%) we detected a significant enrichment of potentially pathogenic variants as compared to controls. Conclusion: Next-generation sequencing combined with microarrays provides the diagnosis for approximately half of the GJB2 mutation–negative individuals. Usher syndrome was found to be more frequent in the study cohort than anticipated. The conditions in a proportion of individuals with nonsyndromic hearing loss, particularly in the undiagnosed group, may have been caused or modified by an accumulation of unfavorable variants across multiple genes. PMID:24875298

  9. Impact of Gene Patents and Licensing Practices on Access to Genetic Testing for Hearing Loss

    PubMed Central

    Chandrasekharan, Subhashini; Fiffer, Melissa

    2011-01-01

    Genetic testing for heritable hearing loss involves a mix of patented and unpatented genes, mutations and testing methods. More than half of all hearing loss is linked to inherited mutations, and five genes are most commonly tested in the United States. There are no patents on three of these genes, but Athena Diagnostics holds exclusive licenses to test for a common mutation in the GJB2 gene associated with about 50% of all cases, as well as mutations in the MTRNR1 gene. This fragmented intellectual property landscape made hearing loss a useful case study for assessing whether patent rights in genetic testing can proliferate or overlap, and whether it is possible to gather the rights necessary to perform testing. Testing for hearing loss is widely available, primarily from academic medical centers. Based on literature reviews and interviews with researchers, research on the genetics of hearing loss has generally not been impeded by patents. There is no consistent evidence of a premium in testing prices attributable to patent status. Athena Diagnostics has, however, used its intellectual property to discourage other providers from offering some tests. There is no definitive answer about the suitability of current patenting and licensing of commonly tested genes because of continuing legal uncertainty about the extent of enforcement of patent rights. Clinicians have also expressed concerns that multiplex tests will be difficult to develop because of overlapping intellectual property and conflict with Athena’s sole provider business model. PMID:20393307

  10. Susceptibility of outer hair cells to cholesterol chelator 2-hydroxypropyl-β-cyclodextrine is prestin-dependent.

    PubMed

    Takahashi, Satoe; Homma, Kazuaki; Zhou, Yingjie; Nishimura, Shinichi; Duan, Chongwen; Chen, Jessie; Ahmad, Aisha; Cheatham, Mary Ann; Zheng, Jing

    2016-01-01

    Niemann-Pick type C1 disease (NPC1) is a fatal genetic disorder caused by impaired intracellular cholesterol trafficking. Recent studies reported ototoxicity of 2-hydroxypropyl- β-cyclodextrin (HPβCD), a cholesterol chelator and the only promising treatment for NPC1. Because outer hair cells (OHCs) are the only cochlear cells affected by HPβCD, we investigated whether prestin, an OHC-specific motor protein, might be involved. Single, high-dose administration of HPβCD resulted in OHC death in prestin wildtype (WT) mice whereas OHCs were largely spared in prestin knockout (KO) mice in the basal region, implicating prestin's involvement in ototoxicity of HPβCD. We found that prestin can interact with cholesterol in vitro, suggesting that HPβCD-induced ototoxicity may involve disruption of this interaction. Time-lapse analysis revealed that OHCs isolated from WT animals rapidly deteriorated upon HPβCD treatment while those from prestin-KOs tolerated the same regimen. These results suggest that a prestin-dependent mechanism contributes to HPβCD ototoxicity. PMID:26903308

  11. A gene for autosomal dominant hearing loss on the short arm of chromosome 1

    SciTech Connect

    Van Camp, G.; Coucke, P.; Willems, P.J.

    1994-09-01

    Hearing loss is the most common form of sensory impairment and many cases are attributable to genetic causes. The genetic defects underlying several syndromic forms of deafness have been identified, but little is known about the causes of non-syndromic hereditary deafness which accounts for the majority of inherited hearing loss. We report here a large Indonesian family with non-syndromal postlingual hearing loss starting in the high frequencies and showing autosomal dominant inheritance. To locate the gene responsible for the hearing loss in this family, we performed a genome search by genetic linkage analysis with microsatellite markers distributed over the whole genome. We have mapped the gene causing deafness in an extended Indonesian family to chromosome 1p with a multipoint lod score higher than 7. Two other smaller families, showing a similar hereditary hearing loss, were also tested for linkage with chromosome 1p. One family originating from the U.S. was linked to this new locus with a multipoint lod score exceeding 5. In another family from the Netherlands this locus was excluded. The flanking markers D1S255 and D1S211 define a region of 6 cM on chromosome 1p which is likely to contain the deafness gene present in the Indonesian and American family.

  12. Expression of prestin-homologous solute carrier (SLC26) in auditory organs of nonmammalian vertebrates and insects.

    PubMed

    Weber, Thomas; Gopfert, Martin C; Winter, Harald; Zimmermann, Ulrike; Kohler, Hanni; Meier, Alexandra; Hendrich, Oliver; Rohbock, Karin; Robert, Daniel; Knipper, Marlies

    2003-06-24

    Prestin, the fifth member of the anion transporter family SLC26, is the outer hair cell molecular motor thought to be responsible for active mechanical amplification in the mammalian cochlea. Active amplification is present in a variety of other auditory systems, yet the prevailing view is that prestin is a motor molecule unique to mammalian ears. Here we identify prestin-related SLC26 proteins that are expressed in the auditory organs of nonmammalian vertebrates and insects. Sequence comparisons revealed the presence of SLC26 proteins in fish (Danio, GenBank accession no. AY278118, and Anguilla, GenBank accession no. BAC16761), mosquitoes (Anopheles, GenBank accession nos. EAA07232 and EAA07052), and flies (Drosophila, GenBank accession no. AAF49285). The fly and zebrafish homologues were cloned and, by using in situ hybridization, shown to be expressed in the auditory organs. In mosquitoes, in turn, the expression of prestin homologues was demonstrated for the auditory organ by using highly specific riboprobes against rat prestin. We conclude that prestin-related SLC26 proteins are widespread, possibly ancestral, constituents of auditory organs and are likely to serve salient roles in mammals and across taxa. PMID:12782792

  13. Susceptibility of outer hair cells to cholesterol chelator 2-hydroxypropyl-β-cyclodextrine is prestin-dependent

    PubMed Central

    Takahashi, Satoe; Homma, Kazuaki; Zhou, Yingjie; Nishimura, Shinichi; Duan, Chongwen; Chen, Jessie; Ahmad, Aisha; Cheatham, Mary Ann; Zheng, Jing

    2016-01-01

    Niemann-Pick type C1 disease (NPC1) is a fatal genetic disorder caused by impaired intracellular cholesterol trafficking. Recent studies reported ototoxicity of 2-hydroxypropyl- β-cyclodextrin (HPβCD), a cholesterol chelator and the only promising treatment for NPC1. Because outer hair cells (OHCs) are the only cochlear cells affected by HPβCD, we investigated whether prestin, an OHC-specific motor protein, might be involved. Single, high-dose administration of HPβCD resulted in OHC death in prestin wildtype (WT) mice whereas OHCs were largely spared in prestin knockout (KO) mice in the basal region, implicating prestin’s involvement in ototoxicity of HPβCD. We found that prestin can interact with cholesterol in vitro, suggesting that HPβCD-induced ototoxicity may involve disruption of this interaction. Time-lapse analysis revealed that OHCs isolated from WT animals rapidly deteriorated upon HPβCD treatment while those from prestin-KOs tolerated the same regimen. These results suggest that a prestin-dependent mechanism contributes to HPβCD ototoxicity. PMID:26903308

  14. [The mutation spectrum of the GJB2 gene in Belarussian patients with hearing loss. Results of pilot genetic screening of hearing impairment in newborns].

    PubMed

    Bliznets, E A; Marcul', D N; Khorov, O G; Markova, T G; Poliakov, A V

    2014-02-01

    A total of 111 unrelated probands and their 8 sibs from Grodno oblast (Belarus) with bilateral isolated sensorineural hearing impairment were studied for the presence of mutations in the connexin 26--GJB2gene. Mutations were detected in 51 probands (46% of the sample). A significantly higher frequency of the GJB2gene mutations was observed in familial cases of the disease with the autosomal recessive type of inheritance (in 78% of families). Detected peculiarities of the GJB2 gene mutation spectrum demonstrated that use of the algorithm, which was developed for Russian patients, is optimal for the molecular study of patients from Be- larus. In the sample of patients with hearing loss, the highest (among other similar samples studied in the world) allele frequency of c.313_326de114 mutation (7% out of all pathological GJB2 alleles) was registered; Polish origin of this deletion was suggested. It was demonstrated that detection of the GJB2 gene mutation on only one patient's chromosome is insufficient to confirm a molecular genetic diagnosis of hearing loss of the DFNB1 genetic type (autosomal recessive hearing loss caused by the GJB2 gene mutations). Pilot screening in the presence of GJB2 gene mutations in newborns from Grodno oblast was conducted. The material from 235 children was studied during the screening; nine heterozygous carriers of the mutation were found. The c.35delG mutation was detected in a homozygous state in a single newborn (hearing loss of moderate severity was subsequently audiologically confirmed in this child). PMID:25711030

  15. The chloride-channel blocker 9-anthracenecarboxylic acid reduces the nonlinear capacitance of prestin-associated charge movement.

    PubMed

    Harasztosi, Csaba; Gummer, Anthony W

    2016-04-01

    The basis of the extraordinary sensitivity and frequency selectivity of the cochlea is a chloride-sensitive protein called prestin which can produce an electromechanical response and which resides in the basolateral plasma membrane of outer hair cells (OHCs). The compound 9-anthracenecarboxylic acid (9-AC), an inhibitor of chloride channels, has been found to reduce the electromechanical response of the cochlea and the OHC mechanical impedance. To elucidate these 9-AC effects, the functional electromechanical status of prestin was assayed by measuring the nonlinear capacitance of OHCs from the guinea-pig cochlea and of prestin-transfected human embryonic kidney 293 (HEK 293) cells. Extracellular application of 9-AC caused reversible, dose-dependent and chloride-sensitive reduction in OHC nonlinear charge transfer, Qmax . Prestin-transfected cells also showed reversible reduction in Qmax . For OHCs, intracellular 9-AC application as well as reduced intracellular pH had no detectable effect on the reduction in Qmax by extracellularly applied 9-AC. In the prestin-transfected cells, cytosolic application of 9-AC approximately halved the blocking efficacy of extracellularly applied 9-AC. OHC inside-out patches presented the whole-cell blocking characteristics. Disruption of the cytoskeleton by preventing actin polymerization with latrunculin A or by decoupling of spectrin from actin with diamide did not affect the 9-AC-evoked reduction in Qmax . We conclude that 9-AC acts on the electromechanical transducer principally by interaction with prestin rather than acting via the cytoskeleton, chloride channels or pH. The 9-AC block presents characteristics in common with salicylate, but is almost an order of magnitude faster. 9-AC provides a new tool for elucidating the molecular dynamics of prestin function. PMID:26869218

  16. A Novel Splice-Site Mutation in the GJB2 Gene Causing Mild Postlingual Hearing Impairment

    PubMed Central

    Gandía, Marta; del Castillo, Francisco J.; Rodríguez-Álvarez, Francisco J.; Garrido, Gema; Villamar, Manuela; Calderón, Manuela; Moreno-Pelayo, Miguel A.; Moreno, Felipe; del Castillo, Ignacio

    2013-01-01

    The DFNB1 subtype of autosomal recessive, nonsyndromic hearing impairment, caused by mutations affecting the GJB2 (connection-26) gene, is highly prevalent in most populations worldwide. DFNB1 hearing impairment is mostly severe or profound and usually appears before the acquisition of speech (prelingual onset), though a small number of hypomorphic missense mutations result in mild or moderate deafness of postlingual onset. We identified a novel GJB2 splice-site mutation, c. -22-2A>C, in three siblings with mild postlingual hearing impairment that were compound heterozygous for c. -22-2A>C and c.35delG. Reverse transcriptase-PCR experiments performed on total RNA extracted from saliva samples from one of these siblings confirmed that c. -22-2A>C abolished the acceptor splice site of the single GJB2 intron, resulting in the absence of normally processed transcripts from this allele. However, we did isolate transcripts from the c. -22-2A>C allele that keep an intact GJB2 coding region and that were generated by use of an alternative acceptor splice site previously unknown. The residual expression of wild-type connection-26 encoded by these transcripts probably underlies the mild severity and late onset of the hearing impairment of these subjects. PMID:24039984

  17. Distinct Expression Patterns Of Causative Genes Responsible For Hereditary Progressive Hearing Loss In Non-Human Primate Cochlea

    PubMed Central

    Hosoya, Makoto; Fujioka, Masato; Ogawa, Kaoru; Okano, Hideyuki

    2016-01-01

    Hearing impairment is the most frequent sensory deficit in humans. Deafness genes, which harbor pathogenic mutations that have been identified in families with hereditary hearing loss, are commonly expressed in the auditory end organ or the cochlea and may contribute to normal hearing function, yet some of the mouse models carrying these mutations fail to recapitulate the hearing loss phenotype. In this study, we find that distinct expression patterns of those deafness genes in the cochlea of a non-human primate, the common marmoset (Callithrix jacchus). We examined 20 genes whose expression in the cochlea has already been reported. The deafness genes GJB3, CRYM, GRHL2, DFNA5, and ATP6B1 were expressed in marmoset cochleae in patterns different from those in mouse cochleae. Of note, all those genes are causative for progressive hearing loss in humans, but not in mice. The other tested genes, including the deafness gene COCH, in which mutation recapitulates deafness in mice, were expressed in a similar manner in both species. The result suggests that the discrepancy in the expression between rodents and primates may account for the phenotypic difference. This limitation of the rodent models can be bypassed by using non-human primate models such as the marmoset. PMID:26915689

  18. Genetic Analysis of Genes Related to Tight Junction Function in the Korean Population with Non-Syndromic Hearing Loss

    PubMed Central

    Sagong, Borum; Cho, Hyun-Ju; Bae, Jae Woong; Kim, Jeongho; Lee, Jinwook; Park, Hong-Joon; Choi, Jae Young; Lee, Kyu-Yup; Kim, Un-Kyung

    2014-01-01

    Tight junctions (TJs) are essential components of eukaryotic cells, and serve as paracellular barriers and zippers between adjacent tissues. TJs are critical for normal functioning of the organ of Corti, a part of the inner ear that causes loss of sensorineural hearing when damaged. To investigate the relation between genes involved in TJ function and hereditary loss of sensorineural hearing in the Korean population, we selected the TJP2 and CLDN14 genes as candidates for gene screening of 135 Korean individuals. The TJP2 gene, mutation of which causes autosomal dominant non-syndromic hearing loss (ADNSHL), lies at the DFNA51 locus on chromosome 9. The CLDN14 gene, mutation of which causes autosomal recessive non-syndromic hearing loss (ARNSHL), lies at the DFNB29 locus on chromosome 21. In the present study, we conducted genetic analyses of the TJP2 and CLDN14 genes in 87 unrelated patients with ADNSHL and 48 unrelated patients with either ARNSHL or potentially sporadic hearing loss. We identified two pathogenic variations, c.334G>A (p.A112T) and c.3562A>G (p.T1188A), and ten single nucleotide polymorphisms (SNPs) in the TJP2 gene. We found eight non-pathogenic variations in the CLDN14 gene. These findings indicate that, whereas mutation of the TJP2 gene might cause ADNSHL, CLDN14 is not a major causative gene for ARNSHL in the Korean population studied. Our findings may improve the understanding of the genetic cause of non-syndromic hearing loss in the Korean population. PMID:24752540

  19. Comprehensive Analysis of Deafness Genes in Families with Autosomal Recessive Nonsyndromic Hearing Loss

    PubMed Central

    Atik, Tahir; Onay, Huseyin; Aykut, Ayca; Bademci, Guney; Kirazli, Tayfun; Tekin, Mustafa; Ozkinay, Ferda

    2015-01-01

    Comprehensive genetic testing has the potential to become the standard of care for individuals with hearing loss. In this study, we investigated the genetic etiology of autosomal recessive nonsyndromic hearing loss (ARNSHL) in a Turkish cohort including individuals with cochlear implant, who had a pedigree suggestive of an autosomal recessive inheritance. A workflow including prescreening of GJB2 and a targeted next generation sequencing panel (Illumına TruSightTM Exome) covering 2761 genes that we briefly called as mendelian exome sequencing was used. This panel includes 102 deafness genes and a number of genes causing Mendelian disorders. Using this approach, we identified causative variants in 21 of 29 families. Three different GJB2 variants were present in seven families. Remaining 14 families had 15 different variants in other known NSHL genes (MYO7A, MYO15A, MARVELD2, TMIE, DFNB31, LOXHD1, GPSM2, TMC1, USH1G, CDH23). Of these variants, eight are novel. Mutation detection rate of our workflow is 72.4%, confirming the usefulness of targeted sequencing approach in NSHL. PMID:26561413

  20. Hear, Hear!

    ERIC Educational Resources Information Center

    Rittner-Heir, Robbin

    2000-01-01

    Examines the problem of acoustics in school classrooms; the problems it creates for student learning, particularly for students with hearing problems; and the impediments to achieving acceptable acoustical levels for school classrooms. Acoustic guidelines are explored and some remedies for fixing sound problems are highlighted. (GR)

  1. Variations in Multiple Syndromic Deafness Genes Mimic Non-syndromic Hearing Loss

    PubMed Central

    Bademci, G.; Cengiz, F. B.; Foster II, J.; Duman, D.; Sennaroglu, L.; Diaz-Horta, O.; Atik, T.; Kirazli, T.; Olgun, L.; Alper, H.; Menendez, I.; Loclar, I.; Sennaroglu, G.; Tokgoz-Yilmaz, S.; Guo, S.; Olgun, Y.; Mahdieh, N.; Bonyadi, M.; Bozan, N.; Ayral, A.; Ozkinay, F.; Yildirim-Baylan, M.; Blanton, S. H.; Tekin, M.

    2016-01-01

    The genetics of both syndromic (SHL) and non-syndromic hearing loss (NSHL) is characterized by a high degree of genetic heterogeneity. We analyzed whole exome sequencing data of 102 unrelated probands with apparently NSHL without a causative variant in known NSHL genes. We detected five causative variants in different SHL genes (SOX10, MITF, PTPN11, CHD7, and KMT2D) in five (4.9%) probands. Clinical re-evaluation of these probands shows that some of them have subtle syndromic findings, while none of them meets clinical criteria for the diagnosis of the associated syndrome (Waardenburg (SOX10 and MITF), Kallmann (CHD7 and SOX10), Noonan/LEOPARD (PTPN11), CHARGE (CHD7), or Kabuki (KMT2D). This study demonstrates that individuals who are evaluated for NSHL can have pathogenic variants in SHL genes that are not usually considered for etiologic studies. PMID:27562378

  2. Variations in Multiple Syndromic Deafness Genes Mimic Non-syndromic Hearing Loss.

    PubMed

    Bademci, G; Cengiz, F B; Foster Ii, J; Duman, D; Sennaroglu, L; Diaz-Horta, O; Atik, T; Kirazli, T; Olgun, L; Alper, H; Menendez, I; Loclar, I; Sennaroglu, G; Tokgoz-Yilmaz, S; Guo, S; Olgun, Y; Mahdieh, N; Bonyadi, M; Bozan, N; Ayral, A; Ozkinay, F; Yildirim-Baylan, M; Blanton, S H; Tekin, M

    2016-01-01

    The genetics of both syndromic (SHL) and non-syndromic hearing loss (NSHL) is characterized by a high degree of genetic heterogeneity. We analyzed whole exome sequencing data of 102 unrelated probands with apparently NSHL without a causative variant in known NSHL genes. We detected five causative variants in different SHL genes (SOX10, MITF, PTPN11, CHD7, and KMT2D) in five (4.9%) probands. Clinical re-evaluation of these probands shows that some of them have subtle syndromic findings, while none of them meets clinical criteria for the diagnosis of the associated syndrome (Waardenburg (SOX10 and MITF), Kallmann (CHD7 and SOX10), Noonan/LEOPARD (PTPN11), CHARGE (CHD7), or Kabuki (KMT2D). This study demonstrates that individuals who are evaluated for NSHL can have pathogenic variants in SHL genes that are not usually considered for etiologic studies. PMID:27562378

  3. A novel C202F mutation in the connexin26 gene (GJB2) associated with autosomal dominant isolated hearing loss.

    PubMed

    Morlé, L; Bozon, M; Alloisio, N; Latour, P; Vandenberghe, A; Plauchu, H; Collet, L; Edery, P; Godet, J; Lina-Granade, G

    2000-05-01

    Mutations in the GJB2 gene encoding connexin26 (CX26) account for up to 50% of cases of autosomal recessive hearing loss. In contrast, only one GJB2 mutation has been reported to date in an autosomal dominant form of isolated prelingual hearing loss. We report here a novel heterozygous 605G-->T mutation in GJB2 in all affected members of a large family with late childhood onset of autosomal dominant isolated hearing loss. The resulting C202F substitution, which lies in the fourth (M4) transmembrane domain of CX26, may impair connexin oligomerisation. Finally, our study suggests that GJB2 should be screened for heterozygous mutations in patients with autosomal dominant isolated hearing impairment, whatever the severity of the disease. PMID:10807696

  4. PAX3 gene deletion detected by microarray analysis in a girl with hearing loss.

    PubMed

    Drozniewska, Malgorzata; Haus, Olga

    2014-01-01

    Deletions of the PAX3 gene have been rarely reported in the literature. Mutations of this gene are a common cause of Waardenburg syndrome type 1 and 3. We report a 16 year old female presenting hearing loss and normal intellectual development, without major features of Waardenburg syndrome type 1, and without family history of the syndrome. Her phenotype, however, overlaps with features of craniofacial-deafness-hand syndrome. Microarray analysis showed ~862 kb de novo deletion at 2q36.1 including PAX3. The above findings suggest that the rearrangement found in our patient appeared de novo and with high probability is a cause of her phenotype. PMID:24839464

  5. Restoration of hearing in the VGLUT3 knockout mouse using virally mediated gene therapy.

    PubMed

    Akil, Omar; Seal, Rebecca P; Burke, Kevin; Wang, Chuansong; Alemi, Aurash; During, Matthew; Edwards, Robert H; Lustig, Lawrence R

    2012-07-26

    Mice lacking the vesicular glutamate transporter-3 (VGLUT3) are congenitally deaf due to loss of glutamate release at the inner hair cell afferent synapse. Cochlear delivery of VGLUT3 using adeno-associated virus type 1 (AAV1) leads to transgene expression in only inner hair cells (IHCs), despite broader viral uptake. Within 2 weeks of AAV1-VGLUT3 delivery, auditory brainstem response (ABR) thresholds normalize, along with partial rescue of the startle response. Lastly, we demonstrate partial reversal of the morphologic changes seen within the afferent IHC ribbon synapse. These findings represent a successful restoration of hearing by gene replacement in mice, which is a significant advance toward gene therapy of human deafness. PMID:22841313

  6. Mutation Analysis of the Common Deafness Genes in Patients with Nonsyndromic Hearing Loss in Linyi by SNPscan Assay

    PubMed Central

    Zhang, Fengguo; Xu, Lei; Zhang, Xue; Zhang, Guodong; Li, Jianfeng; Lv, Huaiqing; Bai, Xiaohui; Wang, Haibo

    2016-01-01

    Hearing loss is a common sensory disorder, and at least 50% of cases are due to a genetic etiology. Although hundreds of genes have been reported to be associated with nonsyndromic hearing loss, GJB2, SLC26A4, and mtDNA12SrRNA are the major contributors. However, the mutation spectrum of these common deafness genes varies among different ethnic groups. The present work summarized mutations in these three genes and their prevalence in 339 patients with nonsyndromic hearing loss at three different special education schools and one children's hospital in Linyi, China. A new multiplex genetic screening system “SNPscan assay” was employed to detect a total of 115 mutations of the above three genes. Finally, 48.67% of the patients were identified with hereditary hearing loss caused by mutations in GJB2, SLC26A4, and mtDNA12SrRNA. The carrying rate of mutations in the three genes was 37.76%, 19.75%, and 4.72%, respectively. This mutation profile in our study is distinct from other parts of China, with high mutation rate of GJB2 suggesting a unique mutation spectrum in this area. PMID:27247933

  7. PON2 and ATP2B2 gene polymorphisms with noise-induced hearing loss

    PubMed Central

    Li, Xiuting; Cao, Jinglian; Wang, Jun; Song, Haiyan; Ji, Guixiang; Dong, Qiu; Wei, Chunlong; Cao, Ying; Wang, Boshen

    2016-01-01

    Background Noise-induced hearing loss (NIHL) is a complex disease induced by a combination of genetic and environmental factors. Paraoxonase2 (PON2) gene involved in the regulation of reactive oxygen species, and affecting the vulnerability of cochlea to NIHL, and ATPase, calcium-transporting, plasma membrane 2 (ATP2B2) gene which encodes plasma membrane calcium-transporting ATPase isoform 2 (PMCA2) are the candidate genes relating to the attack of NIHL. In this study, we investigated whether ATP2B2 and PON2 polymorphisms were associated with NIHL in Chinese of Han nationality population. Methods We performed a case-control study between six single nucleotide polymorphisms (SNPs) (rs1719571, rs3209637 and rs4327369 within ATP2B2, rs12026, rs7785846 and rs12704796 within PON2) and NIHL in 454 subjects. All the SNPs were genotypes, using the TaqMan MGB probe assay. Odds ratios (ORs) were calculated with 95% confidence intervals (95% CIs) with logistic regression analysis to test the level of association for SNPs. Results In our study, 221 subjects with hearing loss and 233 subjects without hearing loss were recruited. The frequencies of the CG and CG + GG genotype of rs12026 (PON2) conferred risk factors for NIHL with adjusted OR values of 2.62 (95% CI, 1.69–4.06) and 2.48 (95% CI, 1.63–3.78), respectively. This kind of significance was also found at locus rs7785846, where genotypes CT and CT + TT were the risk types, with adjusted ORs of 2.52 (95% CI, 1.62–3.93) and 2.35 (95% CI, 1.54–3.58), respectively. We performed stratified analysis per noise exposure level, when it came to rs7785846 and rs12026 in the >92 dB(A) noise exposure group, the subjects who carried heterozygote were of significantly (P<0.01) higher susceptibility to NIHL than homozygote carriers. By contrast, no significantly higher risk was found for any rs12704796 genotypes or any genotypes in ATP2B2 (P>0.05), which may suggest that these SNPs did not have significant effects on noise

  8. A novel frameshift mutation of POU4F3 gene associated with autosomal dominant non-syndromic hearing loss

    SciTech Connect

    Lee, Hee Keun; Park, Hong-Joon; Lee, Kyu-Yup; Park, Rekil; Kim, Un-Kyung

    2010-06-04

    Autosomal dominant mutations in the transcription factor POU4F3 gene are associated with non-syndromic hearing loss in humans; however, there have been few reports of mutations in this gene worldwide. We performed a mutation analysis of the POU4F3 gene in 42 unrelated Koreans with autosomal dominant non-syndromic hearing loss, identifying a novel 14-bp deletion mutation in exon 2 (c.662del14) in one patient. Audiometric examination revealed severe bilateral sensorineural hearing loss in this patient. The novel mutation led to a truncated protein that lacked both functional POU domains. We further investigated the functional distinction between wild-type and mutant POU4F3 proteins using in vitro assays. The wild-type protein was completely localized in the nucleus, while the truncation of protein seriously affected its nuclear localization. In addition, the mutant failed to activate reporter gene expression. This is the first report of a POU4F3 mutation in Asia, and moreover our data suggest that further investigation will need to delineate ethnicity-specific genetic background for autosomal dominant non-syndromic hearing loss within Asian populations.

  9. Sudden Sensorineural Hearing Loss and Polymorphisms in Iron Homeostasis Genes: New Insights from a Case-Control Study

    PubMed Central

    Ciorba, Andrea; Aimoni, Claudia; Orioli, Elisa; Zeri, Giulia; Vigliano, Marco; Gemmati, Donato

    2015-01-01

    Background. Even if various pathophysiological events have been proposed as explanations, the putative cause of sudden hearing loss remains unclear. Objectives. To investigate and to reveal associations (if any) between the main iron-related gene variants and idiopathic sudden sensorineural hearing loss. Study Design. Case-control study. Materials and Methods. A total of 200 sudden sensorineural hearing loss patients (median age 63.65 years; range 10–92) were compared with 400 healthy control subjects. The following genetic variants were investigated: the polymorphism c.−8CG in the promoter of the ferroportin gene (FPN1; SLC40A1), the two isoforms C1 and C2 (p.P570S) of the transferrin protein (TF), the amino acidic substitutions p.H63D and p.C282Y in the hereditary hemochromatosis protein (HFE), and the polymorphism c.–582AG in the promoter of the HEPC gene, which encodes the protein hepcidin (HAMP). Results. The homozygous genotype c.−8GG of the SLC40A1 gene revealed an OR for ISSNHL risk of 4.27 (CI 95%, 2.65–6.89; P = 0.001), being overrepresented among cases. Conclusions. Our study indicates that the homozygous genotype FPN1 −8GG was significantly associated with increased risk of developing sudden hearing loss. These findings suggest new research should be conducted in the field of iron homeostasis in the inner ear. PMID:25789325

  10. Genetic counseling for patients with nonsyndromic hearing impairment directed by gene analysis

    PubMed Central

    MA, DINGYUAN; ZHANG, JINGJING; LUO, CHUNYU; LIN, YING; JI, XIUQING; HU, PING; XU, ZHENGFENG

    2016-01-01

    The aim of the present study was to investigate the genetic etiology of patients with nonsyndromic hearing impairment through gene analysis, and provide accurate genetic counseling and prenatal diagnosis for deaf patients and families with deaf children. Previous molecular etiological studies have demonstrated that the most common molecular changes in Chinese patients with nonsyndromic hearing loss (NSHL) involved gap junction protein β 2, solute carrier family 26, member 4 (SLC26A4), and mitochondrial DNA 12S rRNA. A total of 117 unrelated NSHL patients were included. Mutation screening was performed by Sanger sequencing in GJB2, 12S rRNA, and the hot-spot regions of SLC26A4. In addition, patients with a single mutation of SLC26A4 in the hot-spot regions underwent complete exon sequencing to identify a mutation in the other allele. A total of 36 of the 117 deaf patients were confirmed to have two pathogenic mutations, which included 4 deaf couples, husband or wife in 11 deaf couples and 17 deaf individuals. In addition, prenatal diagnoses was performed in 7 pregnant women at 18–21 weeks gestation who had previously given birth to a deaf child, and the results showed that two fetal genotypes were the same as the proband's genotypes, four fetuses carried one pathogenic gene from their parents, and one fetus was identified to have no mutations. Taken together, the genetic testing of deaf patients can provide reasonable guidance to deaf patients and families with deaf children. PMID:26783197

  11. Glutamate transporter homolog-based model predicts that anion-π interaction is the mechanism for the voltage-dependent response of prestin.

    PubMed

    Lovas, Sándor; He, David Z Z; Liu, Huizhan; Tang, Jie; Pecka, Jason L; Hatfield, Marcus P D; Beisel, Kirk W

    2015-10-01

    Prestin is the motor protein of cochlear outer hair cells. Its unique capability to perform direct, rapid, and reciprocal electromechanical conversion depends on membrane potential and interaction with intracellular anions. How prestin senses the voltage change and interacts with anions are still unknown. Our three-dimensional model of prestin using molecular dynamics simulations predicts that prestin contains eight transmembrane-spanning segments and two helical re-entry loops and that tyrosyl residues are the structural specialization of the molecule for the unique function of prestin. Using site-directed mutagenesis and electrophysiological techniques, we confirmed that residues Tyr(367), Tyr(486), Tyr(501), and Tyr(508) contribute to anion binding, interacting with intracellular anions through novel anion-π interactions. Such weak interactions, sensitive to voltage and mechanical stimulation, confer prestin with a unique capability to perform electromechanical and mechanoelectric conversions with exquisite sensitivity. This novel mechanism is completely different from all known mechanisms seen in ion channels, transporters, and motor proteins. PMID:26283790

  12. A missense variant of the ATP1A2 gene is associated with a novel phenotype of progressive sensorineural hearing loss associated with migraine.

    PubMed

    Oh, Se-Kyung; Baek, Jeong-In; Weigand, Karl M; Venselaar, Hanka; Swarts, Herman G P; Park, Seong-Hyun; Hashim Raza, Muhammad; Jung, Da Jung; Choi, Soo-Young; Lee, Sang-Heun; Friedrich, Thomas; Vriend, Gert; Koenderink, Jan B; Kim, Un-Kyung; Lee, Kyu-Yup

    2015-05-01

    Hereditary sensorineural hearing loss is an extremely clinical and genetic heterogeneous disorder in humans. Especially, syndromic hearing loss is subdivided by combinations of various phenotypes, and each subtype is related to different genes. We present a new form of progressive hearing loss with migraine found to be associated with a variant in the ATP1A2 gene. The ATP1A2 gene has been reported as the major genetic cause of familial migraine by several previous studies. A Korean family presenting progressive hearing loss with migraine was ascertained. The affected members did not show any aura or other neurologic symptoms during migraine attacks, indicating on a novel phenotype of syndromic hearing loss. To identify the causative gene, linkage analysis and whole-exome sequencing were performed. A novel missense variant, c.571G>A (p.(Val191Met)), was identified in the ATP1A2 gene that showed co-segregation with the phenotype in the family. In silico studies suggest that this variant causes a change in hydrophobic interactions and thereby slightly destabilize the A-domain of Na(+)/K(+)-ATPase. However, functional studies failed to show any effect of the p.(Val191Met) substitution on the catalytic rate of this enzyme. We describe a new phenotype of progressive hearing loss with migraine associated with a variant in the ATP1A2 gene. This study suggests that a variant in Na(+)/K(+)-ATPase can be involved in both migraine and hearing loss. PMID:25138102

  13. Diversity of the causal genes in hearing impaired Algerian individuals identified by whole exome sequencing.

    PubMed

    Ammar-Khodja, Fatima; Bonnet, Crystel; Dahmani, Malika; Ouhab, Sofiane; Lefèvre, Gaelle M; Ibrahim, Hassina; Hardelin, Jean-Pierre; Weil, Dominique; Louha, Malek; Petit, Christine

    2015-05-01

    The genetic heterogeneity of congenital hearing disorders makes molecular diagnosis expensive and time-consuming using conventional techniques such as Sanger sequencing of DNA. In order to design an appropriate strategy of molecular diagnosis in the Algerian population, we explored the diversity of the involved mutations by studying 65 families affected by autosomal recessive forms of nonsyndromic hearing impairment (DFNB forms), which are the most prevalent early onset forms. We first carried out a systematic screening for mutations in GJB2 and the recurrent p.(Arg34*) mutation in TMC1, which were found in 31 (47.7%) families and 1 (1.5%) family, respectively. We then performed whole exome sequencing in nine of the remaining families, and identified the causative mutations in all the patients analyzed, either in the homozygous state (eight families) or in the compound heterozygous state (one family): (c.709C>T: p.(Arg237*)) and (c.2122C>T: p.(Arg708*)) in OTOF, (c.1334T>G: p.(Leu445Trp)) in SLC26A4, (c.764T>A: p.(Met255Lys)) in GIPC3, (c.518T>A: p.(Cys173Ser)) in LHFPL5, (c.5336T>C: p.(Leu1779Pro)) in MYO15A, (c.1807G>T: p.(Val603Phe)) in OTOA, (c.6080dup: p.(Asn2027Lys*9)) in PTPRQ, and (c.6017del: p.(Gly2006Alafs*13); c.7188_7189ins14: p.(Val2397Leufs*2)) in GPR98. Notably, 7 of these 10 mutations affecting 8 different genes had not been reported previously. These results highlight for the first time the genetic heterogeneity of the early onset forms of nonsyndromic deafness in Algerian families. PMID:26029705

  14. Diversity of the causal genes in hearing impaired Algerian individuals identified by whole exome sequencing

    PubMed Central

    Ammar-Khodja, Fatima; Bonnet, Crystel; Dahmani, Malika; Ouhab, Sofiane; Lefèvre, Gaelle M; Ibrahim, Hassina; Hardelin, Jean-Pierre; Weil, Dominique; Louha, Malek; Petit, Christine

    2015-01-01

    The genetic heterogeneity of congenital hearing disorders makes molecular diagnosis expensive and time-consuming using conventional techniques such as Sanger sequencing of DNA. In order to design an appropriate strategy of molecular diagnosis in the Algerian population, we explored the diversity of the involved mutations by studying 65 families affected by autosomal recessive forms of nonsyndromic hearing impairment (DFNB forms), which are the most prevalent early onset forms. We first carried out a systematic screening for mutations in GJB2 and the recurrent p.(Arg34*) mutation in TMC1, which were found in 31 (47.7%) families and 1 (1.5%) family, respectively. We then performed whole exome sequencing in nine of the remaining families, and identified the causative mutations in all the patients analyzed, either in the homozygous state (eight families) or in the compound heterozygous state (one family): (c.709C>T: p.(Arg237*)) and (c.2122C>T: p.(Arg708*)) in OTOF, (c.1334T>G: p.(Leu445Trp)) in SLC26A4, (c.764T>A: p.(Met255Lys)) in GIPC3, (c.518T>A: p.(Cys173Ser)) in LHFPL5, (c.5336T>C: p.(Leu1779Pro)) in MYO15A, (c.1807G>T: p.(Val603Phe)) in OTOA, (c.6080dup: p.(Asn2027Lys*9)) in PTPRQ, and (c.6017del: p.(Gly2006Alafs*13); c.7188_7189ins14: p.(Val2397Leufs*2)) in GPR98. Notably, 7 of these 10 mutations affecting 8 different genes had not been reported previously. These results highlight for the first time the genetic heterogeneity of the early onset forms of nonsyndromic deafness in Algerian families. PMID:26029705

  15. PSIP1/LEDGF: a new gene likely involved in sensorineural progressive hearing loss

    PubMed Central

    Girotto, Giorgia; Scheffer, Déborah I.; Morgan, Anna; Vozzi, Diego; Rubinato, Elisa; Di Stazio, Mariateresa; Muzzi, Enrico; Pensiero, Stefano; Giersch, Anne B.; Corey, David P.; Gasparini, Paolo

    2015-01-01

    Hereditary Hearing Loss (HHL) is an extremely heterogeneous disorder. Approximately 30 out of 80 known HHL genes are associated with autosomal dominant forms. Here, we identified PSIP1/LEDGF (isoform p75) as a novel strong candidate gene involved in dominant HHL. Using exome sequencing we found a frameshift deletion (c.1554_1555del leading to p.E518Dfs*2) in an Italian pedigree affected by sensorineural mild-to-moderate HHL but also showing a variable eye phenotype (i.e. uveitis, optic neuropathy). This deletion led to a premature stop codon (p.T519X) with truncation of the last 12 amino acids. PSIP1 was recently described as a transcriptional co-activator regulated by miR-135b in vestibular hair cells of the mouse inner ear as well as a possible protector against photoreceptor degeneration. Here, we demonstrate that it is ubiquitously expressed in the mouse inner ear. The PSIP1 mutation is associated with a peculiar audiometric slope toward the high frequencies. These findings indicate that PSIP1 likely plays an important role in HHL. PMID:26689366

  16. Targeted gene capture and massively parallel sequencing identify TMC1 as the causative gene in a six-generation Chinese family with autosomal dominant hearing loss.

    PubMed

    Gao, Xue; Huang, Sha-Sha; Yuan, Yong-Yi; Wang, Guo-Jian; Xu, Jin-Cao; Ji, Yu-Bin; Han, Ming-Yu; Yu, Fei; Kang, Dong-Yang; Lin, Xi; Dai, Pu

    2015-10-01

    Hereditary nonsyndromic hearing loss is extremely heterogeneous. Mutations in the transmembrane channel-like gene1 (TMC1) are known to cause autosomal dominant and recessive forms of nonsyndromic hearing loss linked to the loci of DFNA36 and DFNB7/11, respectively. We characterized a six-generation Chinese family (5315) with progressive, postlingual autosomal dominant nonsyndromic hearing loss (ADNSHL). By combining targeted capture of 82 known deafness genes, next-generation sequencing and bioinformatic analysis, we identified TMC1 c.1714G>A (p. D572N) as the disease-causing mutation. This mutation co-segregated with hearing loss in other family members and was not detected in 308 normal controls. In order to determine the prevalence of TMC1 c.1714G>A in Chinese ADNSHL families, we used DNA samples from 67 ADNSHL families with sloping audiogram and identified two families carry this mutation. To determine whether it arose from a common ancestor, we analyzed nine STR markers. Our results indicated that TMC1 c.1714G>A (p.D572N) account for about 4.4% (3/68) of ADNSHL in the Chinese population. PMID:26079994

  17. Update of the spectrum of GJB2 gene mutations in 152 Moroccan families with autosomal recessive nonsyndromic hearing loss.

    PubMed

    Bakhchane, Amina; Bousfiha, Amale; Charoute, Hicham; Salime, Sara; Detsouli, Mustapha; Snoussi, Khalid; Nadifi, Sellama; Kabine, Mostafa; Rouba, Hassan; Dehbi, Hind; Roky, Rachida; Charif, Majida; Barakat, Abdelhamid

    2016-06-01

    Deafness is one of the most common genetic diseases in humans and is subject to important genetic heterogeneity. The most common cause of non syndromic hearing loss (NSHL) is mutations in the GJB2 gene. This study aims to update and evaluate the spectrum of GJB2 allele variants in 152 Moroccan multiplex families with non syndromic hearing loss. Seven different mutations were detected: c.35delG, p.V37I, p.E47X, p.G200R, p.Del120E, p.R75Q, the last three mutations were described for the first time in Moroccan deaf patients, in addition to a novel nonsense mutation, the c.385G>T which is not referenced in any database. Sixty six families (43.42%) have mutations in the coding region of GJB2, while the homozygous c.35delG mutation still to date the most represented 51/152 (33.55%). The analysis of the geographical distribution of mutations located in GJB2 gene showed more allelic heterogeneity in the north and center compared to the south of Morocco. Our results showed that the GJB2 gene is a major contributor to non syndromic hearing loss in Morocco. Thus, this report of the GJB2 mutations spectrum all over Morocco has an important implication for establishing a suitable molecular diagnosis. PMID:27169813

  18. Changes in neuronal activity and gene expression in guinea-pig auditory brainstem after unilateral partial hearing loss.

    PubMed

    Dong, S; Mulders, W H A M; Rodger, J; Robertson, D

    2009-03-31

    Spontaneous neural hyperactivity in the central auditory pathway is often associated with deafness, the most common form of which is partial hearing loss. We quantified both peripheral hearing loss and spontaneous activity in single neurons of the contralateral inferior colliculus in a guinea-pig model 1 week after a unilateral partial deafness induced by cochlear mechanical lesion. We also measured mRNA levels of candidate genes in the same animals using quantitative real-time PCR. Spontaneous hyperactivity was most marked in the frequency region of the peripheral hearing loss. Expression of glutamate decarboxylase 1 (GAD1), GABA-A receptor subunit alpha-1 (GABRA1), and potassium channel subfamily K member 15 (KCNK15) was decreased ipsilaterally in the cochlear nucleus and bilaterally in the inferior colliculus. A member of RAB family of small GTPase (RAB3A) was decreased in both ipsilateral cochlear nucleus and contralateral inferior colliculus. RAB3 GTPase activating protein subunit 1 (RAB3GAP1) and glycine receptor subunit alpha-1 (GLRA1) were reduced ipsilaterally in the cochlear nucleus only. These results suggest that a decrease in inhibitory neurotransmission and an increase in membrane excitability may contribute to elevated neuronal spontaneous activity in the auditory brainstem following unilateral partial hearing loss. PMID:19356697

  19. Hearing Loss

    MedlinePlus

    ... version of this page please turn Javascript on. Hearing Loss What is Hearing Loss? Hearing loss is a common problem caused by ... sec Click to watch this video Types of Hearing Loss Hearing loss comes in many forms. It can ...

  20. [Auditory neuropathy due to the Q829X mutation in the gene encoding otoferlin (OTOF) in an infant screened for newborn hearing impairment].

    PubMed

    Gallo-Terán, J; Morales-Angulo, C; Sánchez, N; Manrique, M; Rodríguez-Ballesteros, M; Moreno-Pelayo, M A; Moreno, E; del Castillo, I

    2006-01-01

    We report an infant with auditory neuropathy secondary to the Q829X mutation in the gene encoding otoferlin (OTOF). Included in a universal newborn hearing screening program, the subject passed the otoacoustic emission (OAEs) test. Given that the infant had a familial history of deafness auditory brainstem response (ABR) testing was performed, revealing a profound hearing impairment. The genetic study confirmed that the subject was homozygous for the Q829X mutation in OTOF. The patient underwent a cochlear implant, obtaining satisfactory results. The moderately high prevalence of this mutation in the Spanish population could produce a significant false negative rate in newborn hearing screening programs using OAEs. PMID:17036997

  1. Hereditary Hearing Loss.

    ERIC Educational Resources Information Center

    Tran, LenhAnh P.; Grundfast, Kenneth M.

    1997-01-01

    This article discusses inheritance patterns in hearing loss, epidemiology, clues to genetic causes, locating genes that cause hereditary disorders, genes related to hearing loss disorders in individuals with Usher syndrome, Waardenburg syndrome, Treacher-Collins syndrome, Branchio-oto-renal and Pendred syndromes, and the significance of finding…

  2. Mutational Analysis of EYA1, SIX1 and SIX5 Genes and Strategies for Management of Hearing Loss in Patients with BOR/BO Syndrome

    PubMed Central

    Jeon, Ju Hyun; Baek, Jeong-In; Lee, Won-Sang; Kim, Un-Kyung; Choi, Jae Young

    2013-01-01

    Background Branchio-oto-renal (BOR) or branchio-otic (BO) syndrome is one of the most common forms of autosomal dominant syndromic hearing loss. Mutations in EYA1, SIX1 and SIX5 genes have been associated with BOR syndrome. In this study, clinical and genetic analyses were performed in patients with BOR/BO syndrome focusing on auditory manifestations and rehabilitation. Methods The audiologic manifestations were reviewed in 10 patients with BOR/BO syndrome. The operative findings and hearing outcome were analyzed in patients who underwent middle ear surgeries. The modality and outcome of auditory rehabilitation were evaluated. Genetic analysis was performed for EYA1, SIX1, and SIX5 genes. Results All patients presented with mixed hearing loss. Five patients underwent middle ear surgeries without successful hearing gain. Cochlear implantation performed in two patients resulted in significant hearing improvement. Genetic analysis revealed four novel EYA1 mutations and a large deletion encompassing the EYA1 gene. Conclusions Auditory rehabilitation in BOR/BO syndrome should be individually tailored keeping in mind the high failure rate after middle ear surgeries. Successful outcome can be expected with cochlear implantations in patients with BOR/BO syndrome who cannot benefit from hearing aids. The novel EYA1 mutations may add to the genotypic and phenotypic spectrum of BOR syndrome in the East Asian population. PMID:23840632

  3. Biallelic nonsense mutations in the otogelin-like gene (OTOGL) in a child affected by mild to moderate hearing impairment.

    PubMed

    Bonnet, C; Louha, M; Loundon, N; Michalski, N; Verpy, E; Smagghe, L; Hardelin, J-P; Rouillon, I; Jonard, L; Couderc, R; Gherbi, S; Garabedian, E N; Denoyelle, F; Petit, C; Marlin, S

    2013-09-25

    Hearing impairment is characterized by great genetic heterogeneity. We report the identification, by whole exome sequencing, of two different nonsense mutations (c.1558C>T; p.Gln520 and c.2773C>T; p.Arg925) in the otogelin-like gene (OTOGL), in a child affected by mild to moderate isolated deafness. Parental genotypes allowed us to conclude that these mutations are present in the compound heterozygous state in the patient. In addition, our clinical data establish that the tectorial membrane and/or the outer hair cells are defective in this form of deafness. PMID:23850727

  4. SLC26A4 gene is frequently involved in nonsyndromic hearing impairment with enlarged vestibular aqueduct in Caucasian populations.

    PubMed

    Albert, Sébastien; Blons, Hélène; Jonard, Laurence; Feldmann, Delphine; Chauvin, Pierre; Loundon, Nathalie; Sergent-Allaoui, Annie; Houang, Muriel; Joannard, Alain; Schmerber, Sébastien; Delobel, Bruno; Leman, Jacques; Journel, Hubert; Catros, Hélène; Dollfus, Hélène; Eliot, Marie-Madeleine; David, Albert; Calais, Catherine; Drouin-Garraud, Valérie; Obstoy, Marie-Françoise; Tran Ba Huy, Patrice; Lacombe, Didier; Duriez, Françoise; Francannet, Christine; Bitoun, Pierre; Petit, Christine; Garabédian, Eréa-Noël; Couderc, Rémy; Marlin, Sandrine; Denoyelle, Françoise

    2006-06-01

    Sensorineural hearing loss is the most frequent sensory deficit of childhood and is of genetic origin in up to 75% of cases. It has been shown that mutations of the SLC26A4 (PDS) gene were involved in syndromic deafness characterized by congenital sensorineural hearing impairment and goitre (Pendred's syndrome), as well as in congenital isolated deafness (DFNB4). While the prevalence of SLC26A4 mutations in Pendred's syndrome is clearly established, it remains to be studied in large cohorts of patients with nonsyndromic deafness and detailed clinical informations. In this report, 109 patients from 100 unrelated families, aged from 1 to 32 years (median age: 10 years), with nonsyndromic deafness and enlarged vestibular aqueduct, were genotyped for SLC26A4 using DHPLC molecular screening and sequencing. In all, 91 allelic variants were observed in 100 unrelated families, of which 19 have never been reported. The prevalence of SLC26A4 mutations was 40% (40/100), with biallelic mutation in 24% (24/100), while six families were homozygous. All patients included in this series had documented deafness, associated with EVA and without any evidence of syndromic disease. Among patients with SLC26A4 biallelic mutations, deafness was more severe, fluctuated more than in patients with no mutation. In conclusion, the incidence of SLC26A4 mutations is high in patients with isolated deafness and enlarged vestibular aqueduct and could represent up to 4% of nonsyndromic hearing impairment. SLC26A4 could be the second most frequent gene implicated in nonsyndromic deafness after GJB2, in this Caucasian population. PMID:16570074

  5. Sulfate and thiosulfate inhibit oxalate transport via a dPrestin (Slc26a6)-dependent mechanism in an insect model of calcium oxalate nephrolithiasis.

    PubMed

    Landry, Greg M; Hirata, Taku; Anderson, Jacob B; Cabrero, Pablo; Gallo, Christopher J R; Dow, Julian A T; Romero, Michael F

    2016-01-15

    Nephrolithiasis is one of the most common urinary tract disorders, with the majority of kidney stones composed of calcium oxalate (CaOx). Given its prevalence (US occurrence 10%), it is still poorly understood, lacking progress in identifying new therapies because of its complex etiology. Drosophila melanogaster (fruitfly) is a recently developed model of CaOx nephrolithiasis. Effects of sulfate and thiosulfate on crystal formation were investigated using the Drosophila model, as well as electrophysiological effects on both Drosophila (Slc26a5/6; dPrestin) and mouse (mSlc26a6) oxalate transporters utilizing the Xenopus laevis oocyte heterologous expression system. Results indicate that both transport thiosulfate with a much higher affinity than sulfate Additionally, both compounds were effective at decreasing CaOx crystallization when added to the diet. However, these results were not observed when compounds were applied to Malpighian tubules ex vivo. Neither compound affected CaOx crystallization in dPrestin knockdown animals, indicating a role for principal cell-specific dPrestin in luminal oxalate transport. Furthermore, thiosulfate has a higher affinity for dPrestin and mSlc26a6 compared with oxalate These data indicate that thiosulfate's ability to act as a competitive inhibitor of oxalate via dPrestin, can explain the decrease in CaOx crystallization seen in the presence of thiosulfate, but not sulfate. Overall, our findings predict that thiosulfate or oxalate-mimics may be effective as therapeutic competitive inhibitors of CaOx crystallization. PMID:26538444

  6. New polymorphic mtDNA restriction site in the 12S rRNA gene detected in Tunisian patients with non-syndromic hearing loss

    SciTech Connect

    Mkaouar-Rebai, Emna Tlili, Abdelaziz; Masmoudi, Saber; Charfeddine, Ilhem; Fakhfakh, Faiza

    2008-05-09

    The 12S rRNA gene was shown to be a hot spot for aminoglycoside-induced and non-syndromic hearing loss since several deafness-associated mtDNA mutations were identified in this gene. Among them, we distinguished the A1555G, the C1494T and the T1095C mutations and C-insertion or deletion at position 961. One hundred Tunisian patients with non-syndromic hearing loss and 100 hearing individuals were analysed in this study. A PCR-RFLP analysis with HaeIII restriction enzyme showed the presence of the A1555G mutation in the 12S rRNA gene in only one out of the 100 patients. In addition, PCR-RFLP and radioactive PCR revealed the presence of a new HaeIII polymorphic restriction site in the same gene of 12S rRNA site in 4 patients with non-syndromic hearing loss. UVIDOC-008-XD analyses showed the presence of this new polymorphic restriction site with a variable heteroplasmic rates at position +1517 of the human mitochondrial genome. On the other hand, direct sequencing of the entire mitochondrial 12S rRNA gene in the 100 patients and in 100 hearing individuals revealed the presence of the A750G and A1438G polymorphisms and the absence of the C1494T, T1095C and 961insC mutations in all the tested individuals. Sequencing of the whole mitochondrial genome in the 4 patients showing the new HaeIII polymorphic restriction site revealed only the presence of the A8860G transition in the MT-ATP6 gene and the A4769G polymorphism in the ND2 gene.

  7. Social Health Insurance-Based Simultaneous Screening for 154 Mutations in 19 Deafness Genes Efficiently Identified Causative Mutations in Japanese Hearing Loss Patients.

    PubMed

    Mori, Kentaro; Moteki, Hideaki; Miyagawa, Maiko; Nishio, Shin-Ya; Usami, Shin-Ichi

    2016-01-01

    Sensorineural hearing loss is one of the most common neurosensory disorders in humans. The incidence of SNHL is estimated to be 1 in 500-1000 newborns. In more than half of these patients, the hearing loss is associated with genetic causes. In Japan, genetic testing for the patients with SNHL using the Invader assay to screen for 46 mutations in 13 deafness genes was approved by the Ministry of Health, Labour and Welfare for inclusion in social health insurance coverage in 2012. Furthermore, from August 2015, this genetic testing has been expanded to screen for 154 mutations in 19 deafness genes using targeted genomic enrichment with massively parallel DNA sequencing combined with the Invader assay and TaqMan genotyping. For this study we analyzed 717 unrelated Japanese hearing loss patients. The total allele frequency of 154 mutations in 19 deafness genes was 32.64% (468/1434) and the total numbers of cases associated with at least one mutation was 44.07% (316/717). Among these, we were able to diagnose 212 (30%) patients, indicating that the present screening could efficiently identify causative mutations in hearing loss patients. It is noteworthy that 27 patients (3.8%) had coexistent multiple mutations in different genes. Five of these 27 patients (0.7%, 5/717 overall) were diagnosed with genetic hearing loss affected by concomitant with responsible mutations in more than two different genes. For patients identified with multiple mutations in different genes, it is necessary to consider that several genes might have an impact on their phenotypes. PMID:27627659

  8. Anti-apoptotic gene Bcl2 is required for stapes development and hearing.

    PubMed

    Carpinelli, M R; Wise, A K; Arhatari, B D; Bouillet, P; Manji, S S M; Manning, M G; Cooray, A A; Burt, R A

    2012-01-01

    In this paper we describe novel and specific roles for the apoptotic regulators Bcl2 and Bim in hearing and stapes development. Bcl2 is anti-apoptotic while Bim is pro-apoptotic. Characterization of the auditory systems of mice deficient for these molecules revealed that Bcl2⁻/⁻ mice suffered severe hearing loss. This was conductive in nature and did not affect sensory cells of the inner ear, with cochlear hair cells and neurons present and functional. Bcl2⁻/⁻ mice were found to have a malformed, often monocrural, porous stapes (the small stirrup-shaped bone of the middle ear), but a normally shaped malleus and incus. The deformed stapes was discontinuous with the incus and sometimes fused to the temporal bones. The defect was completely rescued in Bcl2⁻/⁻Bim⁻/⁻ mice and partially rescued in Bcl2⁻/⁻Bim⁺/⁻ mice, which displayed high-frequency hearing loss and thickening of the stapes anterior crus. The Bcl2⁻/⁻ defect arose in utero before or during the cartilage stage of stapes development. These results implicate Bcl2 and Bim in regulating survival of second pharyngeal arch or neural crest cells that give rise to the stapes during embryonic development. PMID:22874999

  9. A Point Mutation in the Gene for Asparagine-Linked Glycosylation 10B (Alg10b) Causes Nonsyndromic Hearing Impairment in Mice (Mus musculus)

    PubMed Central

    Probst, Frank J.; Corrigan, Rebecca R.; del Gaudio, Daniela; Salinger, Andrew P.; Lorenzo, Isabel; Gao, Simon S.; Chiu, Ilene; Xia, Anping

    2013-01-01

    The study of mouse hearing impairment mutants has led to the identification of a number of human hearing impairment genes and has greatly furthered our understanding of the physiology of hearing. The novel mouse mutant neurological/sensory 5 (nse5) demonstrates a significantly reduced or absent startle response to sound and is therefore a potential murine model of human hearing impairment. Genetic analysis of 500 intercross progeny localized the mutant locus to a 524 kilobase (kb) interval on mouse chromosome 15. A missense mutation in a highly-conserved amino acid was found in the asparagine-linked glycosylation 10B gene (Alg10b), which is within the critical interval for the nse5 mutation. A 20.4 kb transgene containing a wildtype copy of the Alg10b gene rescued the mutant phenotype in nse5/nse5 homozygous animals, confirming that the mutation in Alg10b is responsible for the nse5/nse5 mutant phenotype. Homozygous nse5/nse5 mutants had abnormal auditory brainstem responses (ABRs), distortion product otoacoustic emissions (DPOAEs), and cochlear microphonics (CMs). Endocochlear potentials (EPs), on the other hand, were normal. ABRs and DPOAEs also confirmed the rescue of the mutant nse5/nse5 phenotype by the wildtype Alg10b transgene. These results suggested a defect in the outer hair cells of mutant animals, which was confirmed by histologic analysis. This is the first report of mutation in a gene involved in the asparagine (N)-linked glycosylation pathway causing nonsyndromic hearing impairment, and it suggests that the hearing apparatus, and the outer hair cells in particular, are exquisitely sensitive to perturbations of the N-linked glycosylation pathway. PMID:24303013

  10. Association of nuclear and mitochondrial genes with audiological examinations in Iranian patients with nonaminoglycoside antibiotics-induced hearing loss

    PubMed Central

    Balali, Maryam; Kamalidehghan, Behnam; Farhadi, Mohammad; Ahmadipour, Fatemeh; Ashkezari, Mahmoud Dehghani; Hemami, Mohsen Rezaei; Arabzadeh, Hossein; Falah, Masoumeh; Meng, Goh Yong; Houshmand, Massoud

    2016-01-01

    Mitochondrial DNA mutations play an important role in causing sensorineural hearing loss. The purpose of this study was to determine the association of the mitochondrial genes RNR1, MT-TL1, and ND1 as well as the nuclear genes GJB2 and GJB6 with audiological examinations in nonfamilial Iranians with cochlear implants, using polymerase chain reaction, DNA sequencing, and RNA secondary structure analysis. We found that there were no novel mutations in the mitochondrial gene 12S rRNA (MT-RNR1) in patients with and without GJB2 mutation (GJB2+ and GJB2−, respectively), but a total of six polymorphisms were found. No mutations were observed in tRNALeu(UUR) (MT-TL1). Furthermore, eight polymorphisms were found in the mitochondrial ND1 gene. Additionally, no mutations were observed in the nuclear GJB6 gene in patients in the GJB2− and GJB2+ groups. The speech intelligibility rating and category of auditory perception tests were statistically assessed in patients in the GJB2− and GJB2+ groups. The results indicated that there was a significant difference (P<0.05) between the categories of auditory perception score in the GJB2− group compared to that in the GJB2+ group. Successful cochlear implantation was observed among individuals with GJB2 mutations (GJB2+) and mitochondrial polymorphisms compared to those without GJB2 mutations (GJB2−). In conclusion, the outcome of this study suggests that variation in the mitochondrial and nuclear genes may influence the penetrance of deafness. Therefore, further genetic and functional studies are required to help patients in making the best choice for cochlear implants. PMID:26889084

  11. Association of nuclear and mitochondrial genes with audiological examinations in Iranian patients with nonaminoglycoside antibiotics-induced hearing loss.

    PubMed

    Balali, Maryam; Kamalidehghan, Behnam; Farhadi, Mohammad; Ahmadipour, Fatemeh; Ashkezari, Mahmoud Dehghani; Hemami, Mohsen Rezaei; Arabzadeh, Hossein; Falah, Masoumeh; Meng, Goh Yong; Houshmand, Massoud

    2016-01-01

    Mitochondrial DNA mutations play an important role in causing sensorineural hearing loss. The purpose of this study was to determine the association of the mitochondrial genes RNR1, MT-TL1, and ND1 as well as the nuclear genes GJB2 and GJB6 with audiological examinations in nonfamilial Iranians with cochlear implants, using polymerase chain reaction, DNA sequencing, and RNA secondary structure analysis. We found that there were no novel mutations in the mitochondrial gene 12S rRNA (MT-RNR1) in patients with and without GJB2 mutation (GJB2(+) and GJB2(-), respectively), but a total of six polymorphisms were found. No mutations were observed in tRNA(Leu) (() (UUR) ()) (MT-TL1). Furthermore, eight polymorphisms were found in the mitochondrial ND1 gene. Additionally, no mutations were observed in the nuclear GJB6 gene in patients in the GJB2(-) and GJB2(+) groups. The speech intelligibility rating and category of auditory perception tests were statistically assessed in patients in the GJB2(-) and GJB2(+) groups. The results indicated that there was a significant difference (P<0.05) between the categories of auditory perception score in the GJB2(-) group compared to that in the GJB2(+) group. Successful cochlear implantation was observed among individuals with GJB2 mutations (GJB2(+)) and mitochondrial polymorphisms compared to those without GJB2 mutations (GJB2(-)). In conclusion, the outcome of this study suggests that variation in the mitochondrial and nuclear genes may influence the penetrance of deafness. Therefore, further genetic and functional studies are required to help patients in making the best choice for cochlear implants. PMID:26889084

  12. Novel form of X-linked nonsyndromic hearing loss with cochlear malformation caused by a mutation in the type IV collagen gene COL4A6

    PubMed Central

    Rost, Simone; Bach, Elisa; Neuner, Cordula; Nanda, Indrajit; Dysek, Sandra; Bittner, Reginald E; Keller, Alexander; Bartsch, Oliver; Mlynski, Robert; Haaf, Thomas; Müller, Clemens R; Kunstmann, Erdmute

    2014-01-01

    Hereditary hearing loss is the most common human sensorineural disorder. Genetic causes are highly heterogeneous, with mutations detected in >40 genes associated with nonsyndromic hearing loss, to date. Whereas autosomal recessive and autosomal dominant inheritance is prevalent, X-linked forms of nonsyndromic hearing impairment are extremely rare. Here, we present a Hungarian three-generation family with X-linked nonsyndromic congenital hearing loss and the underlying genetic defect. Next-generation sequencing and subsequent segregation analysis detected a missense mutation (c.1771G>A, p.Gly591Ser) in the type IV collagen gene COL4A6 in all affected family members. Bioinformatic analysis and expression studies support this substitution as being causative. COL4A6 encodes the alpha-6 chain of type IV collagen of basal membranes, which forms a heterotrimer with two alpha-5 chains encoded by COL4A5. Whereas mutations in COL4A5 and contiguous X-chromosomal deletions involving COL4A5 and COL4A6 are associated with X-linked Alport syndrome, a nephropathy associated with deafness and cataract, mutations in COL4A6 alone have not been related to any hereditary disease so far. Moreover, our index patient and other affected family members show normal renal and ocular function, which is not consistent with Alport syndrome, but with a nonsyndromic type of hearing loss. In situ hybridization and immunostaining demonstrated expression of the COL4A6 homologs in the otic vesicle of the zebrafish and in the murine inner ear, supporting its role in normal ear development and function. In conclusion, our results suggest COL4A6 as being the fourth gene associated with X-linked nonsyndromic hearing loss. PMID:23714752

  13. Novel form of X-linked nonsyndromic hearing loss with cochlear malformation caused by a mutation in the type IV collagen gene COL4A6.

    PubMed

    Rost, Simone; Bach, Elisa; Neuner, Cordula; Nanda, Indrajit; Dysek, Sandra; Bittner, Reginald E; Keller, Alexander; Bartsch, Oliver; Mlynski, Robert; Haaf, Thomas; Müller, Clemens R; Kunstmann, Erdmute

    2014-02-01

    Hereditary hearing loss is the most common human sensorineural disorder. Genetic causes are highly heterogeneous, with mutations detected in >40 genes associated with nonsyndromic hearing loss, to date. Whereas autosomal recessive and autosomal dominant inheritance is prevalent, X-linked forms of nonsyndromic hearing impairment are extremely rare. Here, we present a Hungarian three-generation family with X-linked nonsyndromic congenital hearing loss and the underlying genetic defect. Next-generation sequencing and subsequent segregation analysis detected a missense mutation (c.1771G>A, p.Gly591Ser) in the type IV collagen gene COL4A6 in all affected family members. Bioinformatic analysis and expression studies support this substitution as being causative. COL4A6 encodes the alpha-6 chain of type IV collagen of basal membranes, which forms a heterotrimer with two alpha-5 chains encoded by COL4A5. Whereas mutations in COL4A5 and contiguous X-chromosomal deletions involving COL4A5 and COL4A6 are associated with X-linked Alport syndrome, a nephropathy associated with deafness and cataract, mutations in COL4A6 alone have not been related to any hereditary disease so far. Moreover, our index patient and other affected family members show normal renal and ocular function, which is not consistent with Alport syndrome, but with a nonsyndromic type of hearing loss. In situ hybridization and immunostaining demonstrated expression of the COL4A6 homologs in the otic vesicle of the zebrafish and in the murine inner ear, supporting its role in normal ear development and function. In conclusion, our results suggest COL4A6 as being the fourth gene associated with X-linked nonsyndromic hearing loss. PMID:23714752

  14. Hearing Aids

    MedlinePlus

    ... more in both quiet and noisy situations. Hearing aids help people who have hearing loss from damage ... your doctor. There are different kinds of hearing aids. They differ by size, their placement on or ...

  15. Hearing Problems

    MedlinePlus

    ... This flow chart will help direct you if hearing loss is a problem for you or a family ... may damage the inner ear. This kind of hearing loss is called OCCUPATIONAL. Prevent occupational hearing loss by ...

  16. Hearing Loss

    MedlinePlus

    ... Devices Can Help? Hearing aids. Hearing aids are electronic, battery-run devices that make sounds louder. There ... to turn up the volume. Cochlear implants. These electronic devices are for people with severe hearing loss. ...

  17. Mutation analysis of the SLC26A4, FOXI1 and KCNJ10 genes in individuals with congenital hearing loss

    PubMed Central

    Pique, Lynn M.; Brennan, Marie-Luise; Davidson, Colin J.; Schaefer, Frederick; Greinwald Jr, John

    2014-01-01

    Pendred syndrome (PDS) and DFNB4 comprise a phenotypic spectrum of sensorineural hearing loss disorders that typically result from biallelic mutations of the SLC26A4 gene. Although PDS and DFNB4 are recessively inherited, sequencing of the coding regions and splice sites of SLC26A4 in individuals suspected to be affected with these conditions often fails to identify two mutations. We investigated the potential contribution of large SLC26A4 deletions and duplications to sensorineural hearing loss (SNHL) by screening 107 probands with one known SLC26A4 mutation by Multiplex Ligation-dependent Probe Amplification (MLPA). A heterozygous deletion, spanning exons 4–6, was detected in only one individual, accounting for approximately 1% of the missing mutations in our cohort. This low frequency is consistent with previously published MLPA results. We also examined the potential involvement of digenic inheritance in PDS/DFNB4 by sequencing the coding regions of FOXI1 and KCNJ10. Of the 29 probands who were sequenced, three carried nonsynonymous variants including one novel sequence change in FOXI1 and two polymorphisms in KCNJ10. We performed a review of prior studies and, in conjunction with our current data, conclude that the frequency of FOXI1 (1.4%) and KCNJ10 (3.6%) variants in PDS/DFNB4 individuals is low. Our results, in combination with previously published reports, indicate that large SLC26A4 deletions and duplications as well as mutations of FOXI1 and KCNJ10 play limited roles in the pathogenesis of SNHL and suggest that other genetic factors likely contribute to the phenotype. PMID:24860705

  18. Molecular biology of hearing

    PubMed Central

    Stöver, Timo; Diensthuber, Marc

    2012-01-01

    The inner ear is our most sensitive sensory organ and can be subdivided into three functional units: organ of Corti, stria vascularis and spiral ganglion. The appropriate stimulus for the organ of hearing is sound, which travels through the external auditory canal to the middle ear where it is transmitted to the inner ear. The inner ear houses the hair cells, the sensory cells of hearing. The inner hair cells are capable of mechanotransduction, the transformation of mechanical force into an electrical signal, which is the basic principle of hearing. The stria vascularis generates the endocochlear potential and maintains the ionic homeostasis of the endolymph. The dendrites of the spiral ganglion form synaptic contacts with the hair cells. The spiral ganglion is composed of neurons that transmit the electrical signals from the cochlea to the central nervous system. In recent years there has been significant progress in research on the molecular basis of hearing. An increasing number of genes and proteins related to hearing are being identified and characterized. The growing knowledge of these genes contributes not only to greater appreciation of the mechanism of hearing but also to a deeper understanding of the molecular basis of hereditary hearing loss. This basic research is a prerequisite for the development of molecular diagnostics and novel therapies for hearing loss. PMID:22558056

  19. Identification of Two Disease-causing Genes TJP2 and GJB2 in a Chinese Family with Unconditional Autosomal Dominant Nonsyndromic Hereditary Hearing Impairment

    PubMed Central

    Wang, Hong-Yang; Zhao, Ya-Li; Liu, Qiong; Yuan, Hu; Gao, Yun; Lan, Lan; Yu, Lan; Wang, Da-Yong; Guan, Jing; Wang, Qiu-Ju

    2015-01-01

    Background: There are more than 300 genetic loci that have been found to be related to hereditary hearing impairment (HHI), including 92 causative genes for nonsyndromic hearing loss, among which 34 genes are related to autosomal dominant nonsyndromic HHI (ADNSHHI). Traditional linkage analysis and candidate gene sequencing are not effective at detecting the ADNSHHI, especially for the unconditional families that may have more than one pathogenic cause. This study identified two disease-causing genes TJP2 and GJB2 in a Chinese family with unconditional ADNSHHI. Methods: To decipher the genetic code of a Chinese family (family 686) with ADNSHHI, different gene screening techniques have been performed, including linkage analysis, candidate genes screening, high-throughput sequencing and Sanger sequencing. These techniques were done on samples obtained from this family over a period of 10 years. Results: We identified a pathogenic missense mutation, c. 2081G>A (p.G694E), in TJP2, a gene that plays a crucial role in apoptosis and age-related hearing loss (ARHL). The mutation was co-segregated in this pedigree in all, but not in the two patients who presented with different phenotypes from the other affected family members. In one of the two patients, we confirmed that the compound heterozygosity for p. Y136* and p.G45E in the GJB2 gene may account for the phenotype shown in this patient. Conclusions: We identified the co-occurrence of two genetic causes in family 686. The possible disease-causing missense mutation of TJP2 in family 686 presents an opportunity for further investigation into ARHL. It is necessary to combine various genes screening methods, especially for some unconventional cases. PMID:26668150

  20. Parallel evolution of auditory genes for echolocation in bats and toothed whales.

    PubMed

    Shen, Yong-Yi; Liang, Lu; Li, Gui-Sheng; Murphy, Robert W; Zhang, Ya-Ping

    2012-06-01

    The ability of bats and toothed whales to echolocate is a remarkable case of convergent evolution. Previous genetic studies have documented parallel evolution of nucleotide sequences in Prestin and KCNQ4, both of which are associated with voltage motility during the cochlear amplification of signals. Echolocation involves complex mechanisms. The most important factors include cochlear amplification, nerve transmission, and signal re-coding. Herein, we screen three genes that play different roles in this auditory system. Cadherin 23 (Cdh23) and its ligand, protocadherin 15 (Pcdh15), are essential for bundling motility in the sensory hair. Otoferlin (Otof) responds to nerve signal transmission in the auditory inner hair cell. Signals of parallel evolution occur in all three genes in the three groups of echolocators--two groups of bats (Yangochiroptera and Rhinolophoidea) plus the dolphin. Significant signals of positive selection also occur in Cdh23 in the Rhinolophoidea and dolphin, and Pcdh15 in Yangochiroptera. In addition, adult echolocating bats have higher levels of Otof expression in the auditory cortex than do their embryos and non-echolocation bats. Cdh23 and Pcdh15 encode the upper and lower parts of tip-links, and both genes show signals of convergent evolution and positive selection in echolocators, implying that they may co-evolve to optimize cochlear amplification. Convergent evolution and expression patterns of Otof suggest the potential role of nerve and brain in echolocation. Our synthesis of gene sequence and gene expression analyses reveals that positive selection, parallel evolution, and perhaps co-evolution and gene expression affect multiple hearing genes that play different roles in audition, including voltage and bundle motility in cochlear amplification, nerve transmission, and brain function. PMID:22761589

  1. [New recurrent extended deletion, including GJB2 and GJB6 genes, results in isolated sensorineural hearing impairment with autosomal recessive type of inheritance].

    PubMed

    Bliznets, E A; Makienko, O N; Okuneva, E G; Markova, T G; Poliakov, A V

    2014-04-01

    Hereditary hearing loss with the autosomal recessive type of inheritance of the DFNB 1 genetic type, caused by mutations in the GJB2 gene, is the main reason of innate non-syndromal hearing impairment in most developed countries of the world (including Russia). Intragenic point mutations prevail among the GJB2 gene defectors; however, extended deletions in the DFNB1 locus are also found with considerable frequency in some populations (for example, Spain, Great Britain, France, United States, and Brazil). Among the four known extended deletions, only one deletion affects directly the GJB2 gene sequence and was described in a single family. A new extended deletion in the GJB2 and GJB6 gene sequences (approximately 101 kb in size; NC_000013.10:g.20,757,021_20,858,394del), detected in three unrelated Russian patients, was described and characterized. Ingush origin of this mutation is assumed. If the new deletion is frequent, its detection is very important for the genetic consulting of families with hereditary hearing impairment. PMID:25715449

  2. Hearing Screening

    ERIC Educational Resources Information Center

    Johnson-Curiskis, Nanette

    2012-01-01

    Hearing levels are threatened by modern life--headsets for music, rock concerts, traffic noises, etc. It is crucial we know our hearing levels so that we can draw attention to potential problems. This exercise requires that students receive a hearing screening for their benefit as well as for making the connection of hearing to listening.

  3. Spectrum and frequency of GJB2, GJB6 and SLC26A4 gene mutations among nonsyndromic hearing loss patients in eastern part of India.

    PubMed

    Adhikary, Bidisha; Ghosh, Sudakshina; Paul, Silpita; Bankura, Biswabandhu; Pattanayak, Arup Kumar; Biswas, Subhradev; Maity, Biswanath; Das, Madhusudan

    2015-12-01

    Genetically caused nonsyndromic hearing loss is highly heterogeneous. Inspite of this large heterogeneity, mutations in the genes GJB2, GJB6 and SLC26A4 are major contributors. The mutation spectrum of these genes varies among different ethnic groups. Only a handful of studies focused on the altered genetic signature of these genes in different demographic regions of India but never focused on the eastern part of the country. Our study for the first time aimed to characterize the mutation profile of these genes in hearing loss patients of West Bengal state, India. Mutations in GJB2, GJB6 and SLC26A4 genes were screened by bidirectional sequencing from 215 congenital nonsyndromic hearing loss patients. Radiological diagnosis was performed in patients with SLC26A4 mutations by temporal bone CT scan. The study revealed that 4.65% and 6.97% patients had monoallelic and biallelic GJB2 mutations respectively. Six mutations were identified, p.W24X being the most frequent one accounting for 71.05% of the mutated alleles. Mutations in GJB6 including the previously identified deletion mutation (GJB6-D13S1830) were not identified in our study. Further, no patients harbored biallelic mutations in the SLC26A4 gene or the common inner ear malformation Enlarged Vestibular Aqueduct (EVA). The mutation profile of GJB2 in our study is distinct from other parts of India, suggesting that the mutation spectrum of this gene varies with ethnicity and geographical origin. The absence of GJB6 mutations and low frequency of SLC26A4 mutations suggest that additional genetic factors may also contribute to this disease. PMID:26188157

  4. Mutation Screening of Exons 7 and 13 of the TMC1 Gene in Autosomal Recessive Non-syndromic Hearing Loss (ARNSHL) in Iran

    PubMed Central

    Moradipour, Negar; Ghasemi-Dehkordi, Payam; Heibati, Fatemeh; Parchami-Barjui, Shahrbanuo; Abolhasani, Marziyeh; Rashki, Ahmad; Hashemzadeh-Chaleshtori, Morteza

    2016-01-01

    Background: Non-syndromic hearing loss (NSHL) is the most common birth defect and occurs in approximately 1/1,000 newborns. NSHL is a heterogeneous trait and can arise due to both genetic and environmental factors. Mutations of the transmembrane channel-like 1 (TMC1) gene cause non-syndromic deafness in humans and mice. Objectives: The aim of the present study was to investigate the association of TMC1 gene mutations of the locus DFNB7/11 in exons 7 and 13 in a cohort of 100 patients with hearing loss in Iran using polymerase chain reaction–single-stranded conformation polymorphism (PCR-SSCP), heteroduplex analysis (HA), and DNA sequencing. Patients and Methods: In this experimental study, the blood samples of 100 NSHL patients were collected from 10 provinces in Iran. These patients had a mean age of 16.5 ± 2.01 years and 74.15% of their parents had consanguinity. DNA was extracted from specimens and mutations of exons 7 and 13 of the TMC1 gene were investigated using PCR-SSCP. All samples were checked via HA reaction and suspected specimens with shift bands were subjected to DNA sequencing for investigation of any gene variation. Results: In this study, no mutation was found in the two exons of TMC1 gene. It was concluded from these results that mutations of the TMC1 gene’s special exons 7 and 13 have a low contribution in patients and are not great of clinical importance in these Iranian provinces. Conclusions: More studies are needed to investigate the relationship between other parts of this gene with hearing loss in different populations through the country. More research could clarify the role of this gene and its relation with deafness and provide essential information for the prevention and management of auditory disorders caused by genetic factors in the Iranian population. PMID:27247785

  5. About Hearing

    MedlinePlus

    ... ability to hear and understand. The duration and nature of a conductive loss will influence a student's ... nih.gov/health/hearing/neuropathy.asp . Implications: The nature and extent to which the hair cells in ...

  6. Aminoglycoside-induced and non-syndromic hearing loss is associated with the G7444A mutation in the mitochondrial COI/tRNA{sup Ser(UCN)} genes in two Chinese families

    SciTech Connect

    Zhu Yi; Liao Zhisu; Li Zhiyuan; Chen Jianfu; Qian Yaping; Tang Xiaowen; Wang Jindan; Yang Li; Li Ronghua; Ji Jinzhang; Choo, Daniel I. |; Lu Jianxin . E-mail: jx@mail.wz.zj.cn; Guan Minxin |||. E-mail: min-xin.guan@chmcc.org

    2006-04-14

    We report here the clinical, genetic, and molecular characterization of two Chinese families with aminoglycoside induced and non-syndromic hearing impairment. Clinical and genetic evaluations revealed the variable severity and age-of-onset in hearing impairment in these families. Strikingly, there were extremely low penetrances of hearing impairment in these Chinese families. Sequence analysis of the complete mitochondrial genomes in these pedigrees showed the distinct sets of mtDNA polymorphism, in addition to the identical G7444A mutation associated with hearing loss. Indeed, the G7444A mutation in the CO1 gene and the precursor of tRNA{sup Ser(UCN)} gene is present in homoplasmy only in the maternal lineage of those pedigrees but not other members of these families and 164 Chinese controls. Their mitochondrial genomes belong to the Eastern Asian haplogroups C5a and D4a, respectively. In fact, the occurrence of the G7444A mutation in these several genetically unrelated subjects affected by hearing impairment strongly indicates that this mutation is involved in the pathogenesis of hearing impairment. However, there was the absence of other functionally significant mtDNA mutations in two Chinese pedigrees carrying the G7444A mutation. Therefore, nuclear modifier gene(s) or aminoglycoside(s) may play a role in the phenotypic expression of the deafness-associated G7444A mutation in these Chinese pedigrees.

  7. Hearing Aids

    MedlinePlus

    ... type and degree of loss. Are there different styles of hearing aids? Styles of hearing aids Source: NIH/NIDCD Behind-the- ... the ear canal and are available in two styles. The in-the-canal (ITC) hearing aid is ...

  8. Validation of Reference Genes for RT–qPCR Analysis in Noise–Induced Hearing Loss: A Study in Wistar Rat

    PubMed Central

    Melgar–Rojas, Pedro; Alvarado, Juan Carlos; Fuentes–Santamaría, Verónica; Gabaldón–Ull, María Cruz; Juiz, José M.

    2015-01-01

    The reverse transcriptase–quantitative polymerase chain reaction (RT–qPCR) requires adequate normalization in order to ensure accurate results. The use of reference genes is the most common method to normalize RT–qPCR assays; however, many studies have reported that the expression of frequently used reference genes is more variable than expected, depending on experimental conditions. Consequently, proper validation of the stability of reference genes is an essential step when performing new gene expression studies. Despite the fact that RT–qPCR has been widely used to elucidate molecular correlates of noise–induced hearing loss (NIHL), up to date there are no reports demonstrating validation of reference genes for the evaluation of changes in gene expression after NIHL. Therefore, in this study we evaluated the expression of some commonly used reference genes (Arbp, b–Act, b2m, CyA, Gapdh, Hprt1, Tbp, Tfrc and UbC) and examined their suitability as endogenous control genes for RT–qPCR analysis in the adult Wistar rat in response to NIHL. Four groups of rats were noise–exposed to generate permanent cochlear damage. Cochleae were collected at different time points after noise exposure and the expression level of candidate reference genes was evaluated by RT–qPCR using geNorm, NormFinder and BestKeeper software to determine expression stability. The three independent applications revealed Tbp as the most stably expressed reference gene. We also suggest a group of top–ranked reference genes that can be combined to obtain suitable reference gene pairs for the evaluation of the effects of noise on gene expression in the cochlea. These findings provide essential basis for further RT–qPCR analysis in studies of NIHL using Wistar rats as animal model. PMID:26366995

  9. Hearing: Noise-Induced Hearing Loss

    MedlinePlus

    MENU Return to Web version Hearing: Noise-Induced Hearing Loss Hearing: Noise-Induced Hearing Loss The importance of hearing Hearing allows you to ... surround the soft tissue of the inner ear. Hearing loss occurs when the inner ear is damaged. What ...

  10. Correspondence regarding Ballana et al., "Mitochondrial 12S rRNA gene mutations affect RNA secondary structure and lead to variable penetrance in hearing impairment".

    PubMed

    Abreu-Silva, R S; Batissoco, A C; Lezirovitz, K; Romanos, J; Rincon, D; Auricchio, M T B M; Otto, P A; Mingroni-Netto, R C

    2006-05-12

    Ballana et al. [E. Ballana, E. Morales, R. Rabionet, B. Montserrat, M. Ventayol, O. Bravo, P. Gasparini, X. Estivill, Mitochondrial 12S rRNA gene mutations affect RNA secondary structure and lead to variable penetrance in hearing impairment, Biochem. Biophys. Res. Commun. 341 (2006) 950-957] detected a T1291C mutation segregating in a Cuban pedigree with hearing impairment. They interpreted it as probably pathogenic, based on family history, RNA conformation prediction and its absence in a control group of 95 Spanish subjects. We screened a sample of 203 deaf subjects and 300 hearing controls (110 "European-Brazilians" and 190 "African-Brazilians") for the mitochondrial mutations A1555G and T1291C. Five deaf subjects had the T1291C substitution, three isolated cases and two familial cases. In the latter, deafness was paternally inherited or segregated with the A1555G mutation. This doesn't support the hypothesis of T1291C mutation being pathogenic. Two "African-Brazilian" controls also had the T1291C substitution. Six of the seven T1291C-carriers (five deaf and two controls) had mitochondrial DNA of African origin, belonging to macrohaplogroup L1/L2. Therefore, these data point to T1291C substitution as most probably an African non-pathogenic polymorphism. PMID:16574076

  11. Screening of DFNB3 in Iranian families with autosomal recessive non-syndromic hearing loss reveals a novel pathogenic mutation in the MyTh4 domain of the MYO15A gene in a linked family

    PubMed Central

    Reiisi, Somayeh; Tabatabaiefar, Mohammad Amin; Sanati, Mohammad Hosein; Chaleshtori, Morteza Hashemzadeh

    2016-01-01

    Objective(s): Non-syndromic sensorineural hearing loss (NSHL) is a common disorder affecting approximately 1 in 500 newborns. This type of hearing loss is extremely heterogeneous and includes over 100 loci. Mutations in the GJB2 gene have been implicated in about half of autosomal recessive non-syndromic hearing loss (ARNSHL) cases, making this the most common cause of ARNSHL. For the latter form of deafness, most frequent genes proposed include GJB2, SLC26A4, MYO15A, OTOF, and CDH23 worldwide. Materials and Methods: The aim of the present study was to define the role and frequency of MYO15A gene mutation in Iranian families. In this study 30 Iranian families were enrolled with over three deaf children and negative for GJB2. Then linkage analysis was performed by six DFNB3 short tandem repeat markers. Following that, mutation detection accomplished using DNA sequencing. Results: One family (3.33%) showed linkage to DFNB3 and a novel mutation was identified in the MYO15A gene (c.6442T>A): as the disease-causing mutation. Mutation co-segregated with hearing loss in the family but was not present in the 100 ethnicity-matched controls. Conclusion: Our results confirmed that the hearing loss of the linked Iranian family was caused by a novel missense mutation in the MYO15A gene. This mutation is the first to be reported in the world and affects the first MyTH4 domain of the protein.

  12. Expression levels of the BAK1 and BCL2 genes highlight the role of apoptosis in age-related hearing impairment.

    PubMed

    Falah, Masoumeh; Najafi, Mohammad; Houshmand, Massoud; Farhadi, Mohammad

    2016-01-01

    Age-related hearing impairment (ARHI) is a progressive and a common sensory disorder in the elderly and will become an increasingly important clinical problem given the growing elderly population. Apoptosis of cochlear cells is an important factor in animal models of ARHI. As these cells cannot regenerate, their loss leads to irreversible hearing impairment. Identification of molecular mechanisms can facilitate disease prevention and effective treatment. In this study, we compared the expression of the genes BAK1 and BCL2 as two arms of the intrinsic apoptosis pathway between patients with ARHI and healthy subjects. ARHI and healthy subjects were selected after an ear nose throat examination, otoscopic investigation, and pure tone audiometry. RNA was extracted from peripheral blood samples, and relative gene expression levels were measured using quantitative real-time polymerase chain reaction. BAK1 and the BAK1/BCL2 ratio were statistically significantly upregulated in the ARHI subjects. The BAK1/BCL2 ratio was positively correlated with the results of the audiometric tests. Our results indicate that BAK-mediated apoptosis may be a core mechanism in the progression of ARHI in humans, similar to finding in animal models. Moreover, the gene expression changes in peripheral blood samples could be used as a rapid and simple biomarker for early detection of ARHI. PMID:27555755

  13. Virally mediated Kcnq1 gene replacement therapy in the immature scala media restores hearing in a mouse model of human Jervell and Lange-Nielsen deafness syndrome

    PubMed Central

    Chang, Qing; Wang, Jianjun; Li, Qi; Kim, Yeunjung; Zhou, Binfei; Wang, Yunfeng; Li, Huawei; Lin, Xi

    2015-01-01

    Mutations in the potassium channel subunit KCNQ1 cause the human severe congenital deafness Jervell and Lange-Nielsen (JLN) syndrome. We applied a gene therapy approach in a mouse model of JLN syndrome (Kcnq1−/− mice) to prevent the development of deafness in the adult stage. A modified adeno-associated virus construct carrying a Kcnq1 expression cassette was injected postnatally (P0–P2) into the endolymph, which resulted in Kcnq1 expression in most cochlear marginal cells where native Kcnq1 is exclusively expressed. We also found that extensive ectopic virally mediated Kcnq1 transgene expression did not affect normal cochlear functions. Examination of cochlear morphology showed that the collapse of the Reissner’s membrane and degeneration of hair cells (HCs) and cells in the spiral ganglia were corrected in Kcnq1−/− mice. Electrophysiological tests showed normal endocochlear potential in treated ears. In addition, auditory brainstem responses showed significant hearing preservation in the injected ears, ranging from 20 dB improvement to complete correction of the deafness phenotype. Our results demonstrate the first successful gene therapy treatment for gene defects specifically affecting the function of the stria vascularis, which is a major site affected by genetic mutations in inherited hearing loss. PMID:26084842

  14. Virally mediated Kcnq1 gene replacement therapy in the immature scala media restores hearing in a mouse model of human Jervell and Lange-Nielsen deafness syndrome.

    PubMed

    Chang, Qing; Wang, Jianjun; Li, Qi; Kim, Yeunjung; Zhou, Binfei; Wang, Yunfeng; Li, Huawei; Lin, Xi

    2015-08-01

    Mutations in the potassium channel subunit KCNQ1 cause the human severe congenital deafness Jervell and Lange-Nielsen (JLN) syndrome. We applied a gene therapy approach in a mouse model of JLN syndrome (Kcnq1(-/-) mice) to prevent the development of deafness in the adult stage. A modified adeno-associated virus construct carrying a Kcnq1 expression cassette was injected postnatally (P0-P2) into the endolymph, which resulted in Kcnq1 expression in most cochlear marginal cells where native Kcnq1 is exclusively expressed. We also found that extensive ectopic virally mediated Kcnq1 transgene expression did not affect normal cochlear functions. Examination of cochlear morphology showed that the collapse of the Reissner's membrane and degeneration of hair cells (HCs) and cells in the spiral ganglia were corrected in Kcnq1(-/-) mice. Electrophysiological tests showed normal endocochlear potential in treated ears. In addition, auditory brainstem responses showed significant hearing preservation in the injected ears, ranging from 20 dB improvement to complete correction of the deafness phenotype. Our results demonstrate the first successful gene therapy treatment for gene defects specifically affecting the function of the stria vascularis, which is a major site affected by genetic mutations in inherited hearing loss. PMID:26084842

  15. Expression levels of the BAK1 and BCL2 genes highlight the role of apoptosis in age-related hearing impairment

    PubMed Central

    Falah, Masoumeh; Najafi, Mohammad; Houshmand, Massoud; Farhadi, Mohammad

    2016-01-01

    Age-related hearing impairment (ARHI) is a progressive and a common sensory disorder in the elderly and will become an increasingly important clinical problem given the growing elderly population. Apoptosis of cochlear cells is an important factor in animal models of ARHI. As these cells cannot regenerate, their loss leads to irreversible hearing impairment. Identification of molecular mechanisms can facilitate disease prevention and effective treatment. In this study, we compared the expression of the genes BAK1 and BCL2 as two arms of the intrinsic apoptosis pathway between patients with ARHI and healthy subjects. ARHI and healthy subjects were selected after an ear nose throat examination, otoscopic investigation, and pure tone audiometry. RNA was extracted from peripheral blood samples, and relative gene expression levels were measured using quantitative real-time polymerase chain reaction. BAK1 and the BAK1/BCL2 ratio were statistically significantly upregulated in the ARHI subjects. The BAK1/BCL2 ratio was positively correlated with the results of the audiometric tests. Our results indicate that BAK-mediated apoptosis may be a core mechanism in the progression of ARHI in humans, similar to finding in animal models. Moreover, the gene expression changes in peripheral blood samples could be used as a rapid and simple biomarker for early detection of ARHI. PMID:27555755

  16. Localization of a Gene for Autosomal Recessive Distal Renal Tubular Acidosis with Normal Hearing (rdRTA2) to 7q33-34

    PubMed Central

    Karet, Fiona E.; Finberg, Karin E.; Nayir, Ahmet; Bakkaloglu, Aysin; Ozen, Seza; Hulton, Sally A.; Sanjad, Sami A.; Al-Sabban, Essam A.; Medina, Juan F.; Lifton, Richard P.

    1999-01-01

    Summary Failure of distal nephrons to excrete excess acid results in the “distal renal tubular acidoses” (dRTA). Early childhood features of autosomal recessive dRTA include severe metabolic acidosis with inappropriately alkaline urine, poor growth, rickets, and renal calcification. Progressive bilateral sensorineural hearing loss (SNHL) is evident in approximately one-third of patients. We have recently identified mutations in ATP6B1, encoding the B-subunit of the collecting-duct apical proton pump, as a cause of recessive dRTA with SNHL. We now report the results of genetic analysis of 13 kindreds with recessive dRTA and normal hearing. Analysis of linkage and molecular examination of ATP6B1 indicated that mutation in ATP6B1 rarely, if ever, accounts for this phenotype, prompting a genomewide linkage search for loci underlying this trait. The results strongly supported linkage with locus heterogeneity to a segment of 7q33-34, yielding a maximum multipoint LOD score of 8.84 with 68% of kindreds linked. The LOD-3 support interval defines a 14-cM region flanked by D7S500 and D7S688. That 4 of these 13 kindreds do not support linkage to rdRTA2 and ATP6B1 implies the existence of at least one additional dRTA locus. These findings establish that genes causing recessive dRTA with normal and impaired hearing are different, and they identify, at 7q33-34, a new locus, rdRTA2, for recessive dRTA with normal hearing. PMID:10577919

  17. Nanotechnology in Auditory Research: Membrane Electromechanics in Hearing

    PubMed Central

    Araya, Mussie; Brownell, William E.

    2016-01-01

    The soft, thin membranes that envelop all living cells are 2D, nanoscale, fluid assemblies of phospholipids, sterols, proteins and other molecules. Mechanical interactions between these components facilitate membrane function, a key example of which is ion flow mediated by the mechanical opening and closing of channels. Hearing and balance are initiated by the modulation of ion flow through mechanoreceptor channels in stereocilia membranes. Cochlear amplification by the outer hair cell involves modulation of ion movement by the membrane protein prestin. Voltage gated ion channels shape the receptor potential in hair cells and are responsible for the initiation of action potentials that are at the heart of sensory processing in the brain. All three processes require a membrane and their kinetics are modulated by the mechanical (ie. material) properties of the membrane. This chapter reviews the methodology for measuring the mechanics of cellular membranes and introduces a method for examining membrane electromechanics. The approach allows examination of electromechanically mediated interactions between the different molecular species in the membrane that contribute to the biology of hearing and balance. PMID:27259937

  18. Nanotechnology in Auditory Research: Membrane Electromechanics in Hearing.

    PubMed

    Araya, Mussie; Brownell, William E

    2016-01-01

    The soft, thin membranes that envelop all living cells are 2D, nanoscale, fluid assemblies of phospholipids, sterols, proteins, and other molecules. Mechanical interactions between these components facilitate membrane function, a key example of which is ion flow mediated by the mechanical opening and closing of channels. Hearing and balance are initiated by the modulation of ion flow through mechanoreceptor channels in stereocilia membranes. Cochlear amplification by the outer hair cell involves modulation of ion movement by the membrane protein prestin. Voltage-gated ion channels shape the receptor potential in hair cells and are responsible for the initiation of action potentials that are at the heart of sensory processing in the brain. All three processes require a membrane and their kinetics are modulated by the mechanical (i.e., material) properties of the membrane. This chapter reviews the methodology for measuring the mechanics of cellular membranes and introduces a method for examining membrane electromechanics. The approach allows examination of electromechanically mediated interactions between the different molecular species in the membrane that contribute to the biology of hearing and balance. PMID:27259937

  19. Hearing Assistive Technology

    MedlinePlus

    ... for the Public / Hearing and Balance Hearing Assistive Technology Hearing Assistive Technology: FM Systems | Infrared Systems | Induction ... Assistive Technology Systems Solutions What are hearing assistive technology systems (HATS)? Hearing assistive technology systems (HATS) are ...

  20. Evaluation of the contribution of the EYA4 and GRHL2 genes in Korean patients with autosomal dominant non-syndromic hearing loss.

    PubMed

    Kim, Ye-Ri; Kim, Min-A; Sagong, Borum; Bae, Seung-Hyun; Lee, Hyo-Jeong; Kim, Hyung-Jong; Choi, Jae Young; Lee, Kyu-Yup; Kim, Un-Kyung

    2015-01-01

    EYA4 and GRHL2 encode transcription factors that play an important role in regulating many developmental stages. Since EYA4 and GRHL2 were identified as the transcription factors for the DFNA10 and DFNA28, 8 EYA4 mutations and 2 GRHL2 mutations have been reported worldwide. However, these genes have been reported in few studies of the Korean population. In this study, we performed a genetic analysis of EYA4 and GRHL2 in 87 unrelated Korean patients with autosomal dominant non-syndromic hearing loss (NSHL). A total of 4 genetic variants in the EYA4 gene were identified, including the 2 nonsense mutations p.S288X and p.Q393X. The novel mutation p.Q393X (c.1177C>T) resulted in a change in the codon at amino acid position 393 from a glutamine to a stop codon. The p.Q393X allele was predicted to encode a truncated protein lacking the entire C-terminal Eya homolog region (Eya HR), which is essential for the interaction with the transcription factor SIX3. The p.S288X (c.863C>A) mutation was found in a Korean family from a previous study. We analyzed p.S288X-linked microsatellite markers and determined that p.S288X might be a founder mutation and a hotspot mutation in Koreans. In GRHL2, a total of 4 genetic variants were identified, but none were associated with hearing loss in Korean patients. This suggests that GRHL2 may not be a main causal gene for autosomal dominant NSHL in Korean patients. In conclusion, our data provide fundamental information to predict the genotypes of Korean patients diagnosed with autosomal dominant NSHL. PMID:25781927

  1. Evaluation of the Contribution of the EYA4 and GRHL2 Genes in Korean Patients with Autosomal Dominant Non-Syndromic Hearing Loss

    PubMed Central

    Sagong, Borum; Bae, Seung-Hyun; Lee, Hyo-Jeong; Kim, Hyung-Jong; Choi, Jae Young; Lee, Kyu-Yup; Kim, Un-Kyung

    2015-01-01

    EYA4 and GRHL2 encode transcription factors that play an important role in regulating many developmental stages. Since EYA4 and GRHL2 were identified as the transcription factors for the DFNA10 and DFNA28, 8 EYA4 mutations and 2 GRHL2 mutations have been reported worldwide. However, these genes have been reported in few studies of the Korean population. In this study, we performed a genetic analysis of EYA4 and GRHL2 in 87 unrelated Korean patients with autosomal dominant non-syndromic hearing loss (NSHL). A total of 4 genetic variants in the EYA4 gene were identified, including the 2 nonsense mutations p.S288X and p.Q393X. The novel mutation p.Q393X (c.1177C>T) resulted in a change in the codon at amino acid position 393 from a glutamine to a stop codon. The p.Q393X allele was predicted to encode a truncated protein lacking the entire C-terminal Eya homolog region (Eya HR), which is essential for the interaction with the transcription factor SIX3. The p.S288X (c.863C>A) mutation was found in a Korean family from a previous study. We analyzed p.S288X-linked microsatellite markers and determined that p.S288X might be a founder mutation and a hotspot mutation in Koreans. In GRHL2, a total of 4 genetic variants were identified, but none were associated with hearing loss in Korean patients. This suggests that GRHL2 may not be a main causal gene for autosomal dominant NSHL in Korean patients. In conclusion, our data provide fundamental information to predict the genotypes of Korean patients diagnosed with autosomal dominant NSHL. PMID:25781927

  2. Adaptive evolution of tight junction protein claudin-14 in echolocating whales.

    PubMed

    Xu, Huihui; Liu, Yang; He, Guimei; Rossiter, Stephen J; Zhang, Shuyi

    2013-11-10

    Toothed whales and bats have independently evolved specialized ultrasonic hearing for echolocation. Recent findings have suggested that several genes including Prestin, Tmc1, Pjvk and KCNQ4 appear to have undergone molecular adaptations associated with the evolution of this ultrasonic hearing in mammals. Here we studied the hearing gene Cldn14, which encodes the claudin-14 protein and is a member of tight junction proteins that functions in the organ of Corti in the inner ear to maintain a cationic gradient between endolymph and perilymph. Particular mutations in human claudin-14 give rise to non-syndromic deafness, suggesting an essential role in hearing. Our results uncovered two bursts of positive selection, one in the ancestral branch of all toothed whales and a second in the branch leading to the delphinid, phocoenid and ziphiid whales. These two branches are the same as those previously reported to show positive selection in the Prestin gene. Furthermore, as with Prestin, the estimated hearing frequencies of whales significantly correlate with numbers of branch-wise non-synonymous substitutions in Cldn14, but not with synonymous changes. However, in contrast to Prestin, we found no evidence of positive selection in bats. Our findings from Cldn14, and comparisons with Prestin, strongly implicate multiple loci in the acquisition of echolocation in cetaceans, but also highlight possible differences in the evolutionary route to echolocation taken by whales and bats. PMID:23965379

  3. Hearing Impairments

    NASA Astrophysics Data System (ADS)

    Cavender, Anna; Ladner, Richard E.

    For many people with hearing impairments, the degree of hearing loss is only a small aspect of their disability and does not necessarily determine the types of accessibility solutions or accommodations that may be required. For some people, the ability to adjust the audio volume may be sufficient. For others, translation to a signed language may be more appropriate. For still others, access to text alternatives may be the best solution. Because of these differences, it is important for researchers in Web accessibility to understand that people with hearing impairments may have very different cultural-linguistic traditions and personal backgrounds.

  4. Human radiation, experimentation, ethics, and gene therapy. Hearing before the Subcommittee on Energy of the Committee on Science, Space, and Technology, U.S. House of Representatives, One Hundred Third Congress, Second Session, February 10, 1994

    SciTech Connect

    1994-12-31

    The hearing addressed the human radiation experimentation of the Department of Energy (DOE) and the ethics of such treatment and gene therapy. Historical aspects of these subjects and their contributions to advancing current medical treatments are presented. Statements of officials from government, industry and educational institutions are provided along with documents submitted for the record.

  5. Fish Hearing.

    ERIC Educational Resources Information Center

    Blaxter, J. H. S.

    1980-01-01

    Provides related information about hearing in fish, including the sensory stimulus of sound in the underwater environment, mechanoreceptors in fish, pressure perception and the swimbladder, specializations in sound conduction peculiar to certain fish families. Includes numerous figures. (CS)

  6. Hearing Aid

    MedlinePlus

    ... and Food and Drug Administration Staff FDA permits marketing of new laser-based hearing aid with potential ... feeds Follow FDA on Twitter Follow FDA on Facebook View FDA videos on YouTube View FDA photos ...

  7. Hearing Impairment

    MedlinePlus

    ... known as noise-induced hearing loss (NIHL) . Personal music players are among the chief culprits of NIHL ... exposure to high noise levels (such as loud music) over time can cause permanent damage to the ...

  8. Maternally inherited cardiomyopathy and hearing loss associated with a novel mutation in the mitochondrial tRNA{sup Lys} gene (G8363A)

    SciTech Connect

    Santorelli, F.M.; Mak, Suk-Chun; El-Schahawi, M.

    1996-05-01

    A novel G8363A mutation in the mtDNA tRNA{sup Lys} gene was associated, in two unrelated families, with a syndrome consisting of encephalomyopathy, sensorineural hearing loss, and hypertrophic cardiomyopathy. Muscle biopsies from the probands showed mitochondrial proliferation and partial defects of complexes I, III, and IV of the electron-transport chain. The G8363A mutation was very abundant (>95%) in muscle samples from the probands and was less copious in blood from 18 maternal relatives (mean 81.3% {plus_minus} 8.5%). Single-muscle-fiber analysis showed significantly higher levels of mutant genomes in cytochrome c oxidase-negative fibers than in cytochrome c oxidase-positive fibers. The mutation was not found in >200 individuals, including normal controls and patients with other mitochondrial encephalomyopathies, thus fulfilling accepted criteria for pathogenicity. 23 refs., 3 figs., 1 tab.

  9. Hearing Loss in Adults.

    ERIC Educational Resources Information Center

    House, John W.

    1997-01-01

    This article discusses hearing loss in adults. It begins with an explanation of the anatomy of the ear and then explains the three types of hearing loss: conductive hearing loss, sensorineural hearing loss, and mixed conductive-sensorineural hearing loss. Tinnitus, hearing aids, and cochlear implants are also addressed. (CR)

  10. Mutational screening in patients with profound sensorineural hearing loss and neurodevelopmental delay: Description of a novel m.3861A > C mitochondrial mutation in the MT-ND1 gene.

    PubMed

    Ammar, Marwa; Tabebi, Mouna; Sfaihi, Lamia; Alila-Fersi, Olfa; Maalej, Marwa; Felhi, Rahma; Chabchoub, Imen; Keskes, Leila; Hachicha, Mongia; Fakhfakh, Faiza; Mkaouar-Rebai, Emna

    2016-06-10

    Mitochondrial diseases caused by mitochondrial dysfunction are a clinically and genetically, heterogeneous group of disorders involving multiple organs, particularly tissues with high-energy demand. Hearing loss is a recognized symptom of a number of mitochondrial diseases and can result from neuronal or cochlear dysfunction. The tissue affected in this pathology is most probably the cochlear hair cells, which are essential for hearing function since they are responsible for maintaining the ionic gradients necessary for sound signal transduction. Several mitochondrial DNA mutations have been associated with hearing loss and since mitochondria are crucial for the cellular energy supply in many tissues, most of these mtDNA mutations affect several tissues and will cause syndromic hearing loss. In the present study, we described 2 patients with sensorineural hearing loss and neurodevelopmental delay in whom we tested mitochondrial genes described to be associated with syndromic hearing loss. One of these patients showed a novel heteroplasmic mitochondrial mutation m.3861A > C (W185C) which lead to a loss of stability of the ND1 protein since it created a new hydrogen bund between the unique created cystein C185 and the A182 residue. In the second patient, we detected two novel heteroplasmic variations m.12350C > A (T5N) and m.14351T > C (E108G) respectively in the MT-ND5 and the MT-ND6 genes. The TopPred II prediction for the E108G variation revealed a decrease of the hydrophobicity in the mutated MT-ND6. PMID:27155156

  11. Whole Exome Sequencing Reveals Homozygous Mutations in RAI1, OTOF, and SLC26A4 Genes Associated with Nonsyndromic Hearing Loss in Altaian Families (South Siberia)

    PubMed Central

    Karafet, Tatiana M.; Morozov, Igor V.; Mikhalskaia, Valeriia Yu.; Zytsar, Marina V.; Bondar, Alexander A.

    2016-01-01

    Hearing loss (HL) is one of the most common sensorineural disorders and several dozen genes contribute to its pathogenesis. Establishing a genetic diagnosis of HL is of great importance for clinical evaluation of deaf patients and for estimating recurrence risks for their families. Efforts to identify genes responsible for HL have been challenged by high genetic heterogeneity and different ethnic-specific prevalence of inherited deafness. Here we present the utility of whole exome sequencing (WES) for identifying candidate causal variants for previously unexplained nonsyndromic HL of seven patients from four unrelated Altaian families (the Altai Republic, South Siberia). The WES analysis revealed homozygous missense mutations in three genes associated with HL. Mutation c.2168A>G (SLC26A4) was found in one family, a novel mutation c.1111G>C (OTOF) was revealed in another family, and mutation c.5254G>A (RAI1) was found in two families. Sanger sequencing was applied for screening of identified variants in an ethnically diverse cohort of other patients with HL (n = 116) and in Altaian controls (n = 120). Identified variants were found only in patients of Altaian ethnicity (n = 93). Several lines of evidences support the association of homozygosity for discovered variants c.5254G>A (RAI1), c.1111C>G (OTOF), and c.2168A>G (SLC26A4) with HL in Altaian patients. Local prevalence of identified variants implies possible founder effect in significant number of HL cases in indigenous population of the Altai region. Notably, this is the first reported instance of patients with RAI1 missense mutation whose HL is not accompanied by specific traits typical for Smith-Magenis syndrome. Presumed association of RAI1 gene variant c.5254G>A with isolated HL needs to be proved by further experimental studies. PMID:27082237

  12. CAOS-Episodic Cerebellar Ataxia, Areflexia, Optic Atrophy, and Sensorineural Hearing Loss: A Third Allelic Disorder of the ATP1A3 Gene.

    PubMed

    Heimer, Gali; Sadaka, Yair; Israelian, Lori; Feiglin, Ariel; Ruggieri, Alessandra; Marshall, Christian R; Scherer, Stephen W; Ganelin-Cohen, Esther; Marek-Yagel, Dina; Tzadok, Michal; Nissenkorn, Andreea; Anikster, Yair; Minassian, Berge A; Zeev, Bruria Ben

    2015-11-01

    We describe the molecular basis of a distinctive syndrome characterized by infantile stress-induced episodic weakness, ataxia, and sensorineural hearing loss, with permanent areflexia and optic nerve pallor. Whole exome sequencing identified a deleterious heterozygous c.2452 G>A, p.(E818K) variant in the ATP1A3 gene and structural analysis predicted its protein-destabilizing effect. This variant has not been reported in context with rapid-onset dystonia parkinsonism and alternating hemiplegia of childhood, the 2 main diseases associated with ATP1A3. The clinical presentation in the family described here differs categorically from these diseases in age of onset, clinical course, cerebellar over extrapyramidal movement disorder predominance, and peripheral nervous system involvement. While this paper was in review, a highly resembling phenotype was reported in additional patients carrying the same c.2452 G>A variant. Our findings substantiate this variant as the cause of a unique inherited autosomal dominant neurologic syndrome that constitutes a third allelic disease of the ATP1A3 gene. PMID:25895915

  13. Associations Between TGFA/TGFB3/MSX1 Gene Polymorphisms and Congenital Non-Syndromic Hearing Impairment in a Chinese Population.

    PubMed

    Du, Jihong; Deng, Jianhua

    2016-01-01

    BACKGROUND The aim of this study was to investigate whether the TGFA/TGFB3/MSX1 gene polymorphisms and haplotypes lead to individual differences between congenital non-syndromic hearing impairment (NSHI) patients and normal people in a Chinese population and to analyze the risk factors for NSHI. MATERIAL AND METHODS Between December 2010 and September 2014, 343 congenital NSHI patients were recruited as cases, and 272 healthy subjects were recruited as controls. Denaturing high-performance liquid chromatography (DHPLC) was used to identify genotypes, SHEsis software was used to conduct gene linkage disequilibrium and haplotype analyses, and regression analysis was performed to identify risk factors for congenital NSHI. RESULTS The distribution of genotype frequencies and allele frequencies of TGFA rs3771494, TGFB3 rs3917201 and rs2268626, and MSX1 rs3821949 and rs62636562 were significantly different between the case and the control groups (all P<0.05). TGFA/TGFB3/MSX1 gene rs3771494, rs1058213, rs3917201, rs2268626, rs3821949, and rs62636562 haplotype analysis showed that haplotype CCGTAC and TTACGT might be protective factors (both P<0.001), while TTGCGC might be a risk factor for the normal population (P<0.001). The other risk factors include paternal smoking, advanced maternal age, maternal sickness history, maternal contact with pesticides or similar drugs, maternal abortion history, maternal medication history, maternal passive smoking history during pregnancy, rs3771494 CT, rs2268626 CC and TC, and rs3821949 GG and AG genotypes were risk factors (all P<0.05), while maternal vitamin supplements during pregnancy, rs3917201 GA, rs62636562 TT and CT genotypes were protective factors for congenital NSHI (all P<0.05). CONCLUSIONS rs3771494, rs3917201, rs2268626, rs3821949 and rs62636562 might be associated with congenital NSHI. PMID:27356075

  14. Associations Between TGFA/TGFB3/MSX1 Gene Polymorphisms and Congenital Non-Syndromic Hearing Impairment in a Chinese Population

    PubMed Central

    Du, Jihong; Deng, Jianhua

    2016-01-01

    Background The aim of this study was to investigate whether the TGFA/TGFB3/MSX1 gene polymorphisms and haplotypes lead to individual differences between congenital non-syndromic hearing impairment (NSHI) patients and normal people in a Chinese population and to analyze the risk factors for NSHI. Material/Methods Between December 2010 and September 2014, 343 congenital NSHI patients were recruited as cases, and 272 healthy subjects were recruited as controls. Denaturing high-performance liquid chromatography (DHPLC) was used to identify genotypes, SHEsis software was used to conduct gene linkage disequilibrium and haplotype analyses, and regression analysis was performed to identify risk factors for congenital NSHI. Results The distribution of genotype frequencies and allele frequencies of TGFA rs3771494, TGFB3 rs3917201 and rs2268626, and MSX1 rs3821949 and rs62636562 were significantly different between the case and the control groups (all P<0.05). TGFA/TGFB3/MSX1 gene rs3771494, rs1058213, rs3917201, rs2268626, rs3821949, and rs62636562 haplotype analysis showed that haplotype CCGTAC and TTACGT might be protective factors (both P<0.001), while TTGCGC might be a risk factor for the normal population (P<0.001). The other risk factors include paternal smoking, advanced maternal age, maternal sickness history, maternal contact with pesticides or similar drugs, maternal abortion history, maternal medication history, maternal passive smoking history during pregnancy, rs3771494 CT, rs2268626 CC and TC, and rs3821949 GG and AG genotypes were risk factors (all P<0.05), while maternal vitamin supplements during pregnancy, rs3917201 GA, rs62636562 TT and CT genotypes were protective factors for congenital NSHI (all P<0.05). Conclusions rs3771494, rs3917201, rs2268626, rs3821949 and rs62636562 might be associated with congenital NSHI. PMID:27356075

  15. Genome-wide association study identifies nox3 as a critical gene for susceptibility to noise-induced hearing loss.

    PubMed

    Lavinsky, Joel; Crow, Amanda L; Pan, Calvin; Wang, Juemei; Aaron, Ksenia A; Ho, Maria K; Li, Qingzhong; Salehide, Pehzman; Myint, Anthony; Monges-Hernadez, Maya; Eskin, Eleazar; Allayee, Hooman; Lusis, Aldons J; Friedman, Rick A

    2015-04-01

    In the United States, roughly 10% of the population is exposed daily to hazardous levels of noise in the workplace. Twin studies estimate heritability for noise-induced hearing loss (NIHL) of approximately 36%, and strain specific variation in sensitivity has been demonstrated in mice. Based upon the difficulties inherent to the study of NIHL in humans, we have turned to the study of this complex trait in mice. We exposed 5 week-old mice from the Hybrid Mouse Diversity Panel (HMDP) to a 10 kHz octave band noise at 108 dB for 2 hours and assessed the permanent threshold shift 2 weeks post exposure using frequency specific stimuli. These data were then used in a genome-wide association study (GWAS) using the Efficient Mixed Model Analysis (EMMA) to control for population structure. In this manuscript we describe our GWAS, with an emphasis on a significant peak for susceptibility to NIHL on chromosome 17 within a haplotype block containing NADPH oxidase-3 (Nox3). Our peak was detected after an 8 kHz tone burst stimulus. Nox3 mutants and heterozygotes were then tested to validate our GWAS. The mutants and heterozygotes demonstrated a greater susceptibility to NIHL specifically at 8 kHz both on measures of distortion product otoacoustic emissions (DPOAE) and on auditory brainstem response (ABR). We demonstrate that this sensitivity resides within the synaptic ribbons of the cochlea in the mutant animals specifically at 8 kHz. Our work is the first GWAS for NIHL in mice and elucidates the power of our approach to identify tonotopic genetic susceptibility to NIHL. PMID:25880434

  16. Genome-Wide Association Study Identifies Nox3 as a Critical Gene for Susceptibility to Noise-Induced Hearing Loss

    PubMed Central

    Lavinsky, Joel; Crow, Amanda L.; Pan, Calvin; Wang, Juemei; Aaron, Ksenia A.; Ho, Maria K.; Li, Qingzhong; Salehide, Pehzman; Myint, Anthony; Monges-Hernadez, Maya; Eskin, Eleazar; Allayee, Hooman; Lusis, Aldons J.; Friedman, Rick A.

    2015-01-01

    In the United States, roughly 10% of the population is exposed daily to hazardous levels of noise in the workplace. Twin studies estimate heritability for noise-induced hearing loss (NIHL) of approximately 36%, and strain specific variation in sensitivity has been demonstrated in mice. Based upon the difficulties inherent to the study of NIHL in humans, we have turned to the study of this complex trait in mice. We exposed 5 week-old mice from the Hybrid Mouse Diversity Panel (HMDP) to a 10 kHz octave band noise at 108 dB for 2 hours and assessed the permanent threshold shift 2 weeks post exposure using frequency specific stimuli. These data were then used in a genome-wide association study (GWAS) using the Efficient Mixed Model Analysis (EMMA) to control for population structure. In this manuscript we describe our GWAS, with an emphasis on a significant peak for susceptibility to NIHL on chromosome 17 within a haplotype block containing NADPH oxidase-3 (Nox3). Our peak was detected after an 8 kHz tone burst stimulus. Nox3 mutants and heterozygotes were then tested to validate our GWAS. The mutants and heterozygotes demonstrated a greater susceptibility to NIHL specifically at 8 kHz both on measures of distortion product otoacoustic emissions (DPOAE) and on auditory brainstem response (ABR). We demonstrate that this sensitivity resides within the synaptic ribbons of the cochlea in the mutant animals specifically at 8 kHz. Our work is the first GWAS for NIHL in mice and elucidates the power of our approach to identify tonotopic genetic susceptibility to NIHL. PMID:25880434

  17. Mutation of the TBCE gene causes disturbance of microtubules in the auditory nerve and cochlear outer hair cell degeneration accompanied by progressive hearing loss in the pmn/pmn mouse.

    PubMed

    Rak, Kristen; Frenz, Silke; Radeloff, Andreas; Groh, Janos; Jablonka, Sibylle; Martini, Rudolf; Hagen, Rudolf; Mlynski, Robert

    2013-12-01

    The progressive motor neuronopathy (pmn/pmn) mouse, an animal model for a fast developing human motor neuron disorder, is additionally characterized by simultaneous progressive sensorineural hearing loss. The gene defect in the pmn/pmn mouse is localized to a missense mutation in the tubulin-specific chaperone E (TBCE) gene on mouse chromosome 13, which is one of the five tubulin-specific chaperons involved in tubulin folding and dimerization. The missense mutation leads to a disturbance of tubulin structures in the auditory nerve and a progressive outer hair cell loss due to apoptosis, which is accompanied by highly elevated ABR-thresholds and loss of DPOAEs. In addition the TBCE protein is selectively expressed in the outer hair cells and the transcellular processes of the inner pillar cells in the cochlea of control and pmn/pmn mouse. We conclude from our study that the mutation of the TBCE gene affects the auditory nerve and the cochlear hair cells simultaneously, leading to progressive hearing loss. This animal model will give the chance to test possible therapeutic strategies in special forms of hearing loss, in which the auditory nerve and the cochlear hair cells are simultaneously affected. PMID:24120439

  18. Age-related hearing loss

    MedlinePlus

    ... is no known single cause of age-related hearing loss. Most commonly, it is caused by changes in the inner ear that occur as you grow older. Your genes and loud noise (from rock concerts or music headphones) may play a large role. The following ...

  19. Living with Hearing Loss

    MedlinePlus

    ... Issues Special Section: Focus on Communication Living with Hearing Loss Past Issues / Fall 2008 Table of Contents For ... Fast Facts There are two main types of hearing loss. Permanent hearing loss (called sensorineural) usually involves damage ...

  20. Hearing Disorders and Deafness

    MedlinePlus

    ... you from hearing sound at all. What causes hearing loss? Some possibilities are Heredity Diseases such as ear ... noise Aging There are two main types of hearing loss. One happens when your inner ear or auditory ...

  1. Noise and Hearing Protection

    MedlinePlus

    ... particularly because such exposure is avoidable. What causes hearing loss? The ear has three main parts: the outer, ... can I tell if my hearing is damaged? Hearing loss usually develops over a period of several years. ...

  2. Help with Hearing

    MedlinePlus

    ... hearing. This problem can make it more diffi- cult to learn speech sounds and language correctly. Take ... how your child is hearing. See how diffi- cult it is to hear words correctly? Some children ...

  3. Hearing Loss and Older Adults

    MedlinePlus

    ... Home » Health Info » Hearing, Ear Infections, and Deafness Hearing Loss and Older Adults On this page: What is ... about hearing loss and older adults? What is hearing loss? Hearing loss is a sudden or gradual decrease ...

  4. MRPS18CP2 alleles and DEFA3 absence as putative chromosome 8p23.1 modifiers of hearing loss due to mtDNA mutation A1555G in the 12S rRNA gene

    PubMed Central

    Ballana, Ester; Mercader, Josep Maria; Fischel-Ghodsian, Nathan; Estivill, Xavier

    2007-01-01

    Background Mitochondrial DNA (mtDNA) mutations account for at least 5% of cases of postlingual, nonsyndromic hearing impairment. Among them, mutation A1555G is frequently found associated with aminoglycoside-induced and/or nonsyndromic hearing loss in families presenting with extremely variable clinical phenotypes. Biochemical and genetic data have suggested that nuclear background is the main factor involved in modulating the phenotypic expression of mutation A1555G. However, although a major nuclear modifying locus was located on chromosome 8p23.1 and regardless intensive screening of the region, the gene involved has not been identified. Methods With the aim to gain insights into the factors that determine the phenotypic expression of A1555G mutation, we have analysed in detail different genetic and genomic elements on 8p23.1 region (DEFA3 gene absence, CLDN23 gene and MRPS18CP2 pseudogene) in a group of 213 A1555G carriers. Results Family based association studies identified a positive association for a polymorphism on MRPS18CP2 and an overrepresentation of DEFA3 gene absence in the deaf group of A1555G carriers. Conclusion Although none of the factors analysed seem to have a major contribution to the phenotype, our findings provide further evidences of the involvement of 8p23.1 region as a modifying locus for A1555G 12S rRNA gene mutation. PMID:18154640

  5. Sound strategies for hearing restoration.

    PubMed

    Géléoc, Gwenaëlle S G; Holt, Jeffrey R

    2014-05-01

    Hearing loss is the most common sensory deficit in humans, with some estimates suggesting up to 300 million affected individuals worldwide. Both environmental and genetic factors contribute to hearing loss and can cause death of sensory cells and neurons. Because these cells do not regenerate, the damage tends to accumulate, leading to profound deafness. Several biological strategies to restore auditory function are currently under investigation. Owing to the success of cochlear implants, which offer partial recovery of auditory function for some profoundly deaf patients, potential biological therapies must extend hearing restoration to include greater auditory acuity and larger patient populations. Here, we review the latest gene, stem-cell, and molecular strategies for restoring auditory function in animal models and the prospects for translating these approaches into viable clinical therapies. PMID:24812404

  6. Sound Strategies for Hearing Restoration

    PubMed Central

    Géléoc, Gwenaëlle S.G.; Holt, Jeffrey R.

    2014-01-01

    Hearing loss is the most common sensory deficit in humans with some estimates suggesting up to 300 million affected individuals worldwide. Both environmental and genetic factors contribute to hearing loss and can cause death of sensory cells and neurons. Since these cells do not regenerate, the damage tends to accumulate leading to profound deafness. Several biological strategies to restore auditory function are currently under investigation. Due to the success of cochlear implants, which offer partial recovery of auditory function for some profoundly deaf patients, potential biological therapies must extend hearing restoration to include greater auditory acuity and larger patient populations. Here we review the latest gene, stem-cell, and molecular strategies for restoring auditory function in animal models and the prospects for translating these approaches into viable clinical therapies. PMID:24812404

  7. [Hearing preservation: Better hearing with advanced technology].

    PubMed

    Rader, T; Helbig, S; Stöver, T; Baumann, U

    2014-05-01

    Preservation of residual hearing after cochlear implantation allows patients the synergetic use of electric and acoustic stimulation (EAS). The application of specific surgical and therapeutic techniques enables the reduction of inner ear trauma, which leads otherwise to complete hearing loss. Due to simultaneous electric and acoustic stimulation, speech understanding is improved especially in noise. EAS is a well-accepted therapeutic treatment for subjects with profound hearing loss in the higher frequencies and no or mild hearing loss in the low frequencies. Several Manufacturers offer individual soft electrodes specially designed for hearing preservation as well as combined electric-acoustic audio processors. PMID:24782208

  8. The master hearing aid.

    PubMed

    Curran, James R; Galster, Jason A

    2013-06-01

    As early as the 1930s the term Master Hearing Aid (MHA) described a device used in the fitting of hearing aids. In their original form, the MHA was a desktop system that allowed for simulated or actual adjustment of hearing aid components that resulted in a changed hearing aid response. Over the years the MHA saw many embodiments and contributed to a number of rationales for the fitting of hearing aids. During these same years, the MHA was viewed by many as an inappropriate means of demonstrating hearing aids; the audio quality of the desktop systems was often superior to the hearing aids themselves. These opinions and the evolution of the MHA have molded the modern perception of hearing aids and the techniques used in the fitting of hearing aids. This article reports on a history of the MHA and its influence on the fitting of hearing aids. PMID:23686682

  9. The Master Hearing Aid

    PubMed Central

    Curran, James R.

    2013-01-01

    As early as the 1930s the term Master Hearing Aid (MHA) described a device used in the fitting of hearing aids. In their original form, the MHA was a desktop system that allowed for simulated or actual adjustment of hearing aid components that resulted in a changed hearing aid response. Over the years the MHA saw many embodiments and contributed to a number of rationales for the fitting of hearing aids. During these same years, the MHA was viewed by many as an inappropriate means of demonstrating hearing aids; the audio quality of the desktop systems was often superior to the hearing aids themselves. These opinions and the evolution of the MHA have molded the modern perception of hearing aids and the techniques used in the fitting of hearing aids. This article reports on a history of the MHA and its influence on the fitting of hearing aids. PMID:23686682

  10. Vestibular function is associated with residual low-frequency hearing loss in patients with bi-allelic mutations in the SLC26A4 gene.

    PubMed

    Jung, Jinsei; Seo, Young Wook; Choi, Jae Young; Kim, Sung Huhn

    2016-05-01

    DFNB4 is non-syndromic, autosomal recessive type of hearing loss with an enlarged vestibular aqueduct (EVA) caused by mutations in SLC26A4/pendrin. Although the characteristics of hearing loss are well known in DFNB4, vestibular function remains inconclusive. We evaluated the vestibular function of 31 patients with bi-allelic mutations in SLC26A4/pendrin and analyzed genetic, radiological, and audiological correlations with vestibular function. In a caloric test, unilateral and bilateral vestibulopathies were detected in 45.2% and 6.4% of patients, respectively; however, only 22.6% had subjective vertigo symptoms. While vestibular phenotype was not significantly associated with specific mutations in genetic alleles or the sizes of the endolymphatic sac and vestibular aqueduct, a residual hearing threshold at a low frequency (500 Hz) was definitely correlated with vestibular function in DFNB4 (p = 0.005). These findings may indicate that vestibular function in DFNB4 deteriorates unilaterally in ears when hearing loss occurs. In conclusion, DFNB4 shows vestibular dysfunction, which is strongly linked to hearing loss at low frequencies without any allelic or anatomical predisposing factor. PMID:26900070

  11. Absence of GJB2 gene mutations, the GJB6 deletion (GJB6-D13S1830) and four common mitochondrial mutations in nonsyndromic genetic hearing loss in a South African population

    PubMed Central

    Kabahuma, Rosemary I.; Ouyang, Xiaomei; Du, Li Lin; Yan, Denise; Hutchin, Tim; Ramsay, Michele; Penn, Claire; Liu, Xue-Zhong

    2015-01-01

    Objective The purpose of this study was to determine the prevalence of mutations in the GJB2 gene, the GJB6-D13S1830 deletion and the four common mitochondrial mutations (A1555G, A3243G, A7511C and A7445G) in a South African population. Methods Using single-strand conformation polymorphism and direct sequencing for screening GJB2 mutation; Multiplex PCR Amplification for GJB6-D13S1830 deletion and Restriction Fragment-Length Polymorphism (PCR-RFLP) analysis for the four common mtDNA mutations. We screened 182 hearing impaired students to determine the frequency of these mutations in the population. Results None of the reported disease causing mutations in GJB2 nor any novel pathogenic mutations in the coding region were detected, in contrast to the findings among Caucasians. The GJB6-D13S1830 deletion and the mitochondrial mutations were not observed in this group. Conclusion These results suggest that GJB2 may not be a significant deafness gene among sub-Saharan Africans, pointing to other unidentified genes as responsible for nonsyndromic hearing loss in these populations. PMID:21392827

  12. Biophysical Mechanisms Underlying Hearing Loss Associated with a Shortened Tectorial Membrane

    NASA Astrophysics Data System (ADS)

    Oghalai, John S.; Xia, Anping; Liu, Christopher C.; Gao, Simon S.; Applegate, Brian E.; Puria, Sunil; Rousso, Itay; Steele, Charles

    2011-11-01

    The tectorial membrane (TM) connects to the stereociliary bundles of outer hair cells (OHCs). Herein, we summarize key experimental data and modeling analyses that describe how biophysical alterations to these connections underlie hearing loss. The heterozygous C1509G mutation in alpha tectorin produces partial congenital hearing loss that progresses in humans. We engineered this mutation in mice, and histology revealed that the TM was shortened. DIC imaging of freshly-dissected cochlea as well as imaging with optical coherence tomography indicated that the TM is malformed and only stimulates the first row of OHCs. Noise exposure produced acute threshold shifts that fully recovered in Tecta+/+ mice although there was some OHC loss within all three rows at the cochlear base. In contrast, threshold shifts only partially recovered in TectaC1509G/+ mice. This was associated with OHC loss more apically and nearly entirely within the first row. Young's modulus of the TM, measured using atomic force microscopy, was substantially reduced at the middle and basal regions. Both the wild-type and heterozygous conditions were simulated in a computational model. This demonstrated that the normalized stress distribution levels between the TM and the tall cilia were significantly elevated in the middle region of the heterozygous cochlea. Another feature of the TectaC1509G/+ mutation is higher prestin expression within all three rows of OHCs. This increased electricallyevoked movements of the reticular lamina and otoacoustic emissions. Furthermore, electrical stimulation was associated with an increased risk of OHC death as measured by vital dye staining. Together, these findings indicate that uncoupling of the TM from some OHCs not only leads to partial hearing loss, but also puts the OHCs that remain coupled at higher risk. Both the mechanics of the malformed TM and increased electromotility contribute to this higher risk profile.

  13. Noise-Induced Hearing Loss

    MedlinePlus

    ... Info » Hearing, Ear Infections, and Deafness Noise-Induced Hearing Loss On this page: What is noise-induced hearing ... additional information about NIHL? What is noise-induced hearing loss? Every day, we experience sound in our environment, ...

  14. De novo 9 Mb deletion of 6q23.2q24.1 disrupting the gene EYA4 in a patient with sensorineural hearing loss, cardiac malformation, and mental retardation.

    PubMed

    Dutrannoy, Véronique; Klopocki, Eva; Wei, Ran; Bommer, Christiane; Mundlos, Stefan; Graul-Neumann, Luitgard M; Trimborn, Marc

    2009-01-01

    We report on a patient carrying a de novo interstitial deletion of chromosomal region 6q23.2-24.1. Interstitial deletions of 6q are rarely reported in the literature. Indeed, only four patients with interstitial deletions overlapping partially with the deleted region in our patient are described in the literature. The aberration was detected by GTG-banding. The size of the deletion was further refined by array-CGH and subsequently fine mapped by quantitative real-time PCR. The exact size of the deletion and the sequence composition of the breakpoints were determined by breakpoint spanning PCR and subsequent sequencing. The patient presented with microcephaly, short stature, patent ductus arteriosus, sensorineural hearing loss, mental retardation, reduced speech development, and abnormal behaviour. The deletion disrupts the gene EYA4. Mutations within this gene are associated with postlingual sensorineural hearing loss. The sequencing of the breakpoint indicated non homologous end joining as the most likely mechanism leading to the rearrangement. PMID:19576303

  15. Low Iron Diet Increases Susceptibility to Noise-Induced Hearing Loss in Young Rats.

    PubMed

    Yu, Fei; Hao, Shuai; Yang, Bo; Zhao, Yue; Yang, Jun

    2016-01-01

    We evaluated the role of iron deficiency (ID) without anemia on hearing function and cochlear pathophysiology of young rats before and after noise exposure. We used rats at developmental stages as an animal model to induce ID without anemia by dietary iron restriction. We have established this dietary restriction model in the rat that should enable us to study the effects of iron deficiency in the absence of severe anemia on hearing and ribbon synapses. Hearing function was measured on Postnatal Day (PND) 21 after induction of ID using auditory brainstem response (ABR). Then, the young rats were exposed to loud noise on PND 21. After noise exposure, hearing function was again measured. We observed the morphology of ribbon synapses, hair cells and spiral ganglion cells (SGCs), and assessed the expression of myosin VIIa, vesicular glutamate transporter 3 and prestin in the cochlea. ID without anemia did not elevate ABR threshold shifts, but reduced ABR wave I peak amplitude of young rats. At 70, 80, and 90 dB SPL, amplitudes of wave I (3.11 ± 0.96 µV, 3.52 ± 1.31 µV, and 4.37 ± 1.08 µV, respectively) in pups from the ID group were decreased compared to the control (5.92 ± 1.67 µV, 6.53 ± 1.70 µV, and 6.90 ± 1.76 µV, respectively) (p < 0.05). Moreover, ID without anemia did not impair the morphology hair cells and SGCs, but decreased the number of ribbon synapses. Before noise exposure, the mean number of ribbon synapses per inner hair cell (IHC) was significantly lower in the ID group (8.44 ± 1.21) compared to that seen in the control (13.08 ± 1.36) (p < 0.05). In addition, the numbers of ribbon synapses per IHC of young rats in the control (ID group) were 6.61 ± 1.59, 3.07 ± 0.83, 5.85 ± 1.63 and 12.25 ± 1.97 (3.75 ± 1.45, 2.03 ± 1.08, 3.81 ± 1.70 and 4.01 ± 1.65) at 1, 4, 7 and 14 days after noise exposure, respectively. Moreover, ABR thresholds at 4 and 8 kHz in young rats from the ID group were significantly elevated at 7 and 14 days after

  16. Low Iron Diet Increases Susceptibility to Noise-Induced Hearing Loss in Young Rats

    PubMed Central

    Yu, Fei; Hao, Shuai; Yang, Bo; Zhao, Yue; Yang, Jun

    2016-01-01

    We evaluated the role of iron deficiency (ID) without anemia on hearing function and cochlear pathophysiology of young rats before and after noise exposure. We used rats at developmental stages as an animal model to induce ID without anemia by dietary iron restriction. We have established this dietary restriction model in the rat that should enable us to study the effects of iron deficiency in the absence of severe anemia on hearing and ribbon synapses. Hearing function was measured on Postnatal Day (PND) 21 after induction of ID using auditory brainstem response (ABR). Then, the young rats were exposed to loud noise on PND 21. After noise exposure, hearing function was again measured. We observed the morphology of ribbon synapses, hair cells and spiral ganglion cells (SGCs), and assessed the expression of myosin VIIa, vesicular glutamate transporter 3 and prestin in the cochlea. ID without anemia did not elevate ABR threshold shifts, but reduced ABR wave I peak amplitude of young rats. At 70, 80, and 90 dB SPL, amplitudes of wave I (3.11 ± 0.96 µV, 3.52 ± 1.31 µV, and 4.37 ± 1.08 µV, respectively) in pups from the ID group were decreased compared to the control (5.92 ± 1.67 µV, 6.53 ± 1.70 µV, and 6.90 ± 1.76 µV, respectively) (p < 0.05). Moreover, ID without anemia did not impair the morphology hair cells and SGCs, but decreased the number of ribbon synapses. Before noise exposure, the mean number of ribbon synapses per inner hair cell (IHC) was significantly lower in the ID group (8.44 ± 1.21) compared to that seen in the control (13.08 ± 1.36) (p < 0.05). In addition, the numbers of ribbon synapses per IHC of young rats in the control (ID group) were 6.61 ± 1.59, 3.07 ± 0.83, 5.85 ± 1.63 and 12.25 ± 1.97 (3.75 ± 1.45, 2.03 ± 1.08, 3.81 ± 1.70 and 4.01 ± 1.65) at 1, 4, 7 and 14 days after noise exposure, respectively. Moreover, ABR thresholds at 4 and 8 kHz in young rats from the ID group were significantly elevated at 7 and 14 days after

  17. Hearing Aid Assembly

    NASA Technical Reports Server (NTRS)

    Grugel, Richard N. (Inventor)

    2002-01-01

    Progress in hearing aids has come a long way. Yet despite such progress hearing aids are not the perfect answer to many hearing problems. Some adult ears cannot accommodate tightly fitting hearing aids. Mouth movements such as chewing, talking, and athletic or other active endeavors also lead to loosely fitting ear molds. It is well accepted that loosely fitting hearing aids are the cause of feedback noise. Since feedback noise is the most common complaint of hearing aid wearers it has been the subject of various patents. Herein a hearing aid assembly is provided eliminating feedback noise. The assembly includes the combination of a hearing aid with a headset developed to constrict feedback noise.

  18. Living with hearing loss

    MedlinePlus

    ... medlineplus.gov/ency/patientinstructions/000360.htm Living with hearing loss To use the sharing features on this page, please enable JavaScript. If you are living with hearing loss, you know that it takes extra effort to ...

  19. Hearing Loss: Screening Newborns

    MedlinePlus

    ... of this page please turn JavaScript on. Feature: Hearing Loss Screening Newborns Past Issues / Spring 2015 Table of ... of newborns in the U.S. are screened for hearing loss before they leave the hospital. Research improves the ...

  20. Genetics of Hearing Loss

    MedlinePlus

    ... in Latin America Information For... Media Policy Makers Genetics of Hearing Loss Language: English Español (Spanish) Recommend ... of hearing loss in babies is due to genetic causes. There are also a number of things ...

  1. Can Baby Hear?

    MedlinePlus

    ... 000 children born in the United States are deaf or hard-of-hearing. Research shows that early ... to this, the average age of identification for deaf and hearing impaired children was close to three ...

  2. Hearing Disorders and Deafness

    MedlinePlus

    ... If you have trouble hearing, you can get help. Possible treatments include hearing aids, cochlear implants, special training, certain medicines, and surgery. NIH: National Institute on Deafness and Other Communication Disorders

  3. Deafness and Hearing Loss.

    ERIC Educational Resources Information Center

    National Information Center for Children and Youth with Disabilities, Washington, DC.

    This brief overview provides information on the definition, incidence, and characteristics of children with hearing impairments and deafness. The federal definitions of hearing impairment and deafness are provided. The different types of hearing loss are noted, including: (1) conductive (caused by diseases or obstructions in the outer or middle…

  4. The Hearing Mechanism

    ERIC Educational Resources Information Center

    Sherbon, James W.

    1978-01-01

    An examination of the hearing mechanism and some of the factors involved in helping problems may offer encouragement for a regular schedule of hearing maintenance. It may also help music educators to become more aware and understanding of their own and students' hearing as it affects musical behavior. (Author)

  5. Rehabilitation of Hearing.

    ERIC Educational Resources Information Center

    World Federation of the Deaf, Rome (Italy).

    Rehabilitation of hearing is considered in five conference papers. Two papers come from Poland: "Rehabilitation of Hearing in Children 'Deaf' in First 5 Years of Age" by D. Borkowska-Gaertig and others and "Possibilities of Hearing Improvement in Adults with Conservative Methods" by T. Bystrzanowska. Also included are "Re-Education and…

  6. Hearing-aid tester

    NASA Technical Reports Server (NTRS)

    Kessinger, R.; Polhemus, J. T.; Waring, J. G.

    1977-01-01

    Hearing aids are automatically checked by circuit that applies half-second test signal every thirty minutes. If hearing-aid output is distorted, too small, or if battery is too low, a warning lamp is activated. Test circuit is incorporated directly into hearing-aid package.

  7. Hearing Problems in Children

    MedlinePlus

    ... age 6 months. That's because children start learning speech and language long before they talk. Hearing problems can be temporary or permanent. Sometimes, ear infections, injuries or diseases affect hearing. If your child does not hear well, get help. NIH: National ...

  8. ABE. The Hearing Impaired.

    ERIC Educational Resources Information Center

    Carver, L. Sue

    This handbook was written to help teachers of adult basic education (ABE) adapt their teaching methods for hearing impaired persons. Written in a narrative format, the guide covers the following topics: ABE for the hearing impaired, hints for working with the hearing impaired without an interpreter, peer pairing, interpreters in the classroom…

  9. Implementing Hearing Safety

    ERIC Educational Resources Information Center

    Cliffe, Roger

    1978-01-01

    Hearing damage from noise exposure and approaches to implementing hearing safety in school industrial laboratories through noise reduction and protective equipment are discussed. Although all states have not adopted the Occupational Safety and Health Act, teachers should be aware of noise hazards and act to protect hearing. (MF)

  10. Hearing Conservation Medical Program

    NASA Technical Reports Server (NTRS)

    1993-01-01

    Background on hearing impairment is presented including causes and criteria for safe noise levels. The purpose of the Hearing Conservation Program at LeRC is outlined, and the specifics of the Medical Surveillance Program for Hearing Impairment at LeRC are discussed.

  11. Spectrum and Frequency of the GJB2 Gene Pathogenic Variants in a Large Cohort of Patients with Hearing Impairment Living in a Subarctic Region of Russia (the Sakha Republic)

    PubMed Central

    Posukh, Olga L.; Teryutin, Fedor M.; Solovyev, Aisen V.; Klarov, Leonid A.; Romanov, Georgii P.; Gotovtsev, Nyurgun N.; Kozhevnikov, Andrey A.; Kirillina, Elena V.; Sidorova, Oksana G.; Vasilyevа, Lena M.; Fedotova, Elvira E.; Morozov, Igor V.; Bondar, Alexander A.; Solovyevа, Natalya A.; Kononova, Sardana K.; Rafailov, Adyum M.; Sazonov, Nikolay N.; Alekseev, Anatoliy N.; Tomsky, Mikhail I.; Dzhemileva, Lilya U.; Khusnutdinova, Elza K.; Fedorova, Sardana A.

    2016-01-01

    Pathogenic variants in the GJB2 gene, encoding connexin 26, are known to be a major cause of hearing impairment (HI). More than 300 allelic variants have been identified in the GJB2 gene. Spectrum and allelic frequencies of the GJB2 gene vary significantly among different ethnic groups worldwide. Until now, the spectrum and frequency of the pathogenic variants in exon 1, exon 2 and the flanking intronic regions of the GJB2 gene have not been described thoroughly in the Sakha Republic (Yakutia), which is located in a subarctic region in Russia. The complete sequencing of the non-coding and coding regions of the GJB2 gene was performed in 393 patients with HI (Yakuts—296, Russians—51, mixed and other ethnicities—46) and in 187 normal hearing individuals of Yakut (n = 107) and Russian (n = 80) populations. In the total sample (n = 580), we revealed 12 allelic variants of the GJB2 gene, 8 of which were recessive pathogenic variants. Ten genotypes with biallelic recessive pathogenic variants in the GJB2 gene (in a homozygous or a compound heterozygous state) were found in 192 out of 393 patients (48.85%). We found that the most frequent GJB2 pathogenic variant in the Yakut patients was c.-23+1G>A (51.82%) and that the second most frequent was c.109G>A (2.37%), followed by c.35delG (1.64%). Pathogenic variants с.35delG (22.34%), c.-23+1G>A (5.31%), and c.313_326del14 (2.12%) were found to be the most frequent among the Russian patients. The carrier frequencies of the c.-23+1G>A and с.109G>A pathogenic variants in the Yakut control group were 10.20% and 2.80%, respectively. The carrier frequencies of с.35delG and c.101T>C were identical (2.5%) in the Russian control group. We found that the contribution of the GJB2 gene pathogenic variants in HI in the population of the Sakha Republic (48.85%) was the highest among all of the previously studied regions of Asia. We suggest that extensive accumulation of the c.-23+1G>A pathogenic variant in the indigenous Yakut

  12. Research on Hearing and Balance--Current and Future Developments.

    ERIC Educational Resources Information Center

    Snow, James B., Jr.

    1997-01-01

    This article reviews current research that has located disease genes causing hearing impairments, discovered the ability of sensory cells of the inner ear to regenerate, developed vaccines to prevent otitis media, developed programmable hearing aids, improved cochlear implants, and demonstrated the positive effects of physical therapy with balance…

  13. Hearing loss in space

    NASA Technical Reports Server (NTRS)

    Buckey, J. C. Jr; Musiek, F. E.; Kline-Schoder, R.; Clark, J. C.; Hart, S.; Havelka, J.

    2001-01-01

    BACKGROUND: Temporary and, in some cases, permanent hearing loss has been documented after long-duration spaceflights. METHODS: We examined all existing published data on hearing loss after space missions to characterize the losses. RESULTS: Data from Russian missions suggest that the hearing loss, when it occurs, affects mainly mid to high frequencies and that using hearing protection often might prevent the loss. Several significant questions remain about hearing loss in space. While the hearing loss has been presumed to be noise-induced, no clear link has been established between noise exposure and hearing loss during spaceflight. In one documented case of temporary hearing loss from the Shuttle-Mir program, the pattern of loss was atypical for a noise-induced loss. Continuous noise levels that have been measured on the Mir and previous space stations, while above engineering standards, are not at levels usually associated with hearing loss in ground-based studies (which have usually been limited to 8-10 h exposure periods). Attempts to measure hearing in space using threshold-based audiograms have been unsuccessful in both the American and Russian programs due to noise interference with the measurements. CONCLUSIONS: The existing data highlight the need for reliable monitoring of both hearing and noise in long-duration spaceflight.

  14. Genetics of Nonsyndromic Congenital Hearing Loss.

    PubMed

    Egilmez, Oguz Kadir; Kalcioglu, M Tayyar

    2016-01-01

    Congenital hearing impairment affects nearly 1 in every 1000 live births and is the most frequent birth defect in developed societies. Hereditary types of hearing loss account for more than 50% of all congenital sensorineural hearing loss cases and are caused by genetic mutations. HL can be either nonsyndromic, which is restricted to the inner ear, or syndromic, a part of multiple anomalies affecting the body. Nonsyndromic HL can be categorised by mode of inheritance, such as autosomal dominant (called DFNA), autosomal recessive (DFNB), mitochondrial, and X-linked (DFN). To date, 125 deafness loci have been reported in the literature: 58 DFNA loci, 63 DFNB loci, and 4 X-linked loci. Mutations in genes that control the adhesion of hair cells, intracellular transport, neurotransmitter release, ionic hemeostasis, and cytoskeleton of hair cells can lead to malfunctions of the cochlea and inner ear. In recent years, with the increase in studies about genes involved in congenital hearing loss, genetic counselling and treatment options have emerged and increased in availability. This paper presents an overview of the currently known genes associated with nonsyndromic congenital hearing loss and mutations in the inner ear. PMID:26989561

  15. Genetics of Nonsyndromic Congenital Hearing Loss

    PubMed Central

    Egilmez, Oguz Kadir; Kalcioglu, M. Tayyar

    2016-01-01

    Congenital hearing impairment affects nearly 1 in every 1000 live births and is the most frequent birth defect in developed societies. Hereditary types of hearing loss account for more than 50% of all congenital sensorineural hearing loss cases and are caused by genetic mutations. HL can be either nonsyndromic, which is restricted to the inner ear, or syndromic, a part of multiple anomalies affecting the body. Nonsyndromic HL can be categorised by mode of inheritance, such as autosomal dominant (called DFNA), autosomal recessive (DFNB), mitochondrial, and X-linked (DFN). To date, 125 deafness loci have been reported in the literature: 58 DFNA loci, 63 DFNB loci, and 4 X-linked loci. Mutations in genes that control the adhesion of hair cells, intracellular transport, neurotransmitter release, ionic hemeostasis, and cytoskeleton of hair cells can lead to malfunctions of the cochlea and inner ear. In recent years, with the increase in studies about genes involved in congenital hearing loss, genetic counselling and treatment options have emerged and increased in availability. This paper presents an overview of the currently known genes associated with nonsyndromic congenital hearing loss and mutations in the inner ear. PMID:26989561

  16. Hearing Loss in Children: Types of Hearing Loss

    MedlinePlus

    ... the ear to the brain so that our brain pathways are part of our hearing. There are four types of hearing loss: Conductive Hearing Loss Hearing loss caused by something that stops sounds from getting through the outer or middle ear. This type of hearing loss can often ...

  17. 12 CFR 1209.12 - Public hearings; closed hearings.

    Code of Federal Regulations, 2014 CFR

    2014-01-01

    ... 12 Banks and Banking 10 2014-01-01 2014-01-01 false Public hearings; closed hearings. 1209.12... PRACTICE AND PROCEDURE Rules of Practice and Procedure § 1209.12 Public hearings; closed hearings. (a... hearings shall be open to the public, except that the Director, in his discretion, may determine...

  18. 12 CFR 1209.12 - Public hearings; closed hearings.

    Code of Federal Regulations, 2012 CFR

    2012-01-01

    ... 12 Banks and Banking 9 2012-01-01 2012-01-01 false Public hearings; closed hearings. 1209.12... PRACTICE AND PROCEDURE Rules of Practice and Procedure § 1209.12 Public hearings; closed hearings. (a... hearings shall be open to the public, except that the Director, in his discretion, may determine...

  19. Review of salicylate-induced hearing loss, neurotoxicity, tinnitus and neuropathophysiology.

    PubMed

    Sheppard, A; Hayes, S H; Chen, G-D; Ralli, M; Salvi, R

    2014-04-01

    Salicylate's ototoxic properties have been well established, inducing tinnitus and a sensory hearing loss when administered in high doses. Peripherally, acute dosing of salicylate causes frequency dependent reductions in DPOAEs and CAP amplitudes in low (<10 kHz) and high (>20 kHz) frequencies more than mid frequencies (10-20 kHz), which interestingly corresponds to the pitch of behaviourally-matched salicylate-induced tinnitus. Chronic salicylate dosing affects the peripheral system by causing a compensatory temporary enhancement in DPOAE amplitudes and up-regulation of prestin mRNA and protein expression. Despite salicylate's antioxidant properties, cultured cochlea studies indicate it also impairs spiral ganglion neurons (SGNs) by paradoxically causing an upsurge of superoxide radicals leading to apoptosis. Centrally, salicylate alters γ-aminobutyric acid (GABA) and serotonin mediated neurotransmission in the central nervous system (CNS), which results in classical and non-classical auditory regions showing hyperactivity after salicylate administration. In the auditory cortex (AC) and lateral amygdala (LA), neuron characteristic frequencies (CF) shift upward and downward to mid frequencies (10-20 kHz) altering tonotopy following salicylate administration. Additionally, current source density (CSD) analysis showed enhanced current flow into the supergranular layer of the auditory cortex after a high systemic dose of salicylate. In humans, auditory perception changes following salicylate or aspirin, including decreased word discrimination and temporal integration ability. The results of previous studies have partially identified the mechanisms that are involved in salicylate-induced tinnitus and hearing loss, however to date some interactions remain convoluted. This review discusses current knowledge of salicylate ototoxicity and interactions. PMID:24843217

  20. Music and hearing aids.

    PubMed

    Madsen, Sara M K; Moore, Brian C J

    2014-01-01

    The signal processing and fitting methods used for hearing aids have mainly been designed to optimize the intelligibility of speech. Little attention has been paid to the effectiveness of hearing aids for listening to music. Perhaps as a consequence, many hearing-aid users complain that they are not satisfied with their hearing aids when listening to music. This issue inspired the Internet-based survey presented here. The survey was designed to identify the nature and prevalence of problems associated with listening to live and reproduced music with hearing aids. Responses from 523 hearing-aid users to 21 multiple-choice questions are presented and analyzed, and the relationships between responses to questions regarding music and questions concerned with information about the respondents, their hearing aids, and their hearing loss are described. Large proportions of the respondents reported that they found their hearing aids to be helpful for listening to both live and reproduced music, although less so for the former. The survey also identified problems such as distortion, acoustic feedback, insufficient or excessive gain, unbalanced frequency response, and reduced tone quality. The results indicate that the enjoyment of listening to music with hearing aids could be improved by an increase of the input and output dynamic range, extension of the low-frequency response, and improvement of feedback cancellation and automatic gain control systems. PMID:25361601

  1. Music and Hearing Aids

    PubMed Central

    Moore, Brian C. J.

    2014-01-01

    The signal processing and fitting methods used for hearing aids have mainly been designed to optimize the intelligibility of speech. Little attention has been paid to the effectiveness of hearing aids for listening to music. Perhaps as a consequence, many hearing-aid users complain that they are not satisfied with their hearing aids when listening to music. This issue inspired the Internet-based survey presented here. The survey was designed to identify the nature and prevalence of problems associated with listening to live and reproduced music with hearing aids. Responses from 523 hearing-aid users to 21 multiple-choice questions are presented and analyzed, and the relationships between responses to questions regarding music and questions concerned with information about the respondents, their hearing aids, and their hearing loss are described. Large proportions of the respondents reported that they found their hearing aids to be helpful for listening to both live and reproduced music, although less so for the former. The survey also identified problems such as distortion, acoustic feedback, insufficient or excessive gain, unbalanced frequency response, and reduced tone quality. The results indicate that the enjoyment of listening to music with hearing aids could be improved by an increase of the input and output dynamic range, extension of the low-frequency response, and improvement of feedback cancellation and automatic gain control systems. PMID:25361601

  2. Do You Hear What Horton Hears?

    ERIC Educational Resources Information Center

    Snyder, Robert; Johnson, Jordan

    2010-01-01

    "I've never heard of a small speck of dust that is able to yell" says Horton of a sound he hears well (Geisel 1954). It is always valuable to connect science to student's interests and their everyday world--so what better way to teach concepts relating to sound than to read "Horton Hears a Who" by Dr. Seuss? Here the authors present several…

  3. Use of Hearing Aids by Adults with Hearing Loss

    MedlinePlus

    ... Epidemiology Use of Hearing Aids by Adults with Hearing Loss [text version] Note: Higher numbers are better. *This ... 2010 and 2020. The number of persons with hearing loss is calculated based on National Health and Nutrition ...

  4. A Genetically-Encoded YFP Sensor with Enhanced Chloride Sensitivity, Photostability and Reduced pH Interference Demonstrates Augmented Transmembrane Chloride Movement by Gerbil Prestin (SLC26a5)

    PubMed Central

    Zhong, Sheng; Navaratnam, Dhasakumar; Santos-Sacchi, Joseph

    2014-01-01

    Background Chloride is the major anion in cells, with many diseases arising from disordered Cl− regulation. For the non-invasive investigation of Cl− flux, YFP-H148Q and its derivatives chameleon and Cl-Sensor previously were introduced as genetically encoded chloride indicators. Neither the Cl− sensitivity nor the pH-susceptibility of these modifications to YFP is optimal for precise measurements of Cl− under physiological conditions. Furthermore, the relatively poor photostability of YFP derivatives hinders their application for dynamic and quantitative Cl− measurements. Dynamic and accurate measurement of physiological concentrations of chloride would significantly affect our ability to study effects of chloride on cellular events. Methodology/Principal Findings In this study, we developed a series of YFP derivatives to remove pH interference, increase photostability and enhance chloride sensitivity. The final product, EYFP-F46L/Q69K/H148Q/I152L/V163S/S175G/S205V/A206K (monomeric Cl-YFP), has a chloride Kd of 14 mM and pKa of 5.9. The bleach time constant of 175 seconds is over 15-fold greater than wild-type EYFP. We have used the sensor fused to the transmembrane protein prestin (gerbil prestin, SLC26a5), and shown for the first time physiological (mM) chloride flux in HEK cells expressing this protein. This modified fluorescent protein will facilitate investigations of dynamics of chloride ions and their mediation of cell function. Conclusions Modifications to YFP (EYFP-F46L/Q69K/H148Q/I152L/V163S/S175G/S205V/A206K (monomeric Cl-YFP) results in a photostable fluorescent protein that allows measurement of physiological changes in chloride concentration while remaining minimally affected by changes in pH. PMID:24901231

  5. [Inner Ear Hearing Loss].

    PubMed

    Hesse, G

    2016-06-01

    Hearing loss is one of the most dominant handicaps in modern societies, which additionally very often is not realized or not admitted. About one quarter of the general population suffers from inner ear hearing loss and is therefore restricted in communicational skills. Demographic factors like increasing age play an important role as well as environmental influences and an increasing sound and noise exposure especially in leisure activities. Thus borders between a "classical" presbyacusis - if it ever existed - and envirionmentally induced hearing loss disappear. Today restrictions in hearing ability develop earlier in age but at the same time they are detected and diagnosed earlier. This paper can eventually enlighten the wide field of inner ear hearing loss only fragmentarily; therefore mainly new research, findings and developments are reviewed. The first part discusses new aspects of diagnostics of inner ear hearing loss and different etiologies. PMID:27259171

  6. Hearing or speech impairment - resources

    MedlinePlus

    ... resources for information on hearing impairment or speech impairment: Alexander Graham Bell Association for the Deaf and Hard of Hearing -- www.agbell.org American Speech-Language-Hearing Association -- www.asha.org/public Center for ...

  7. Screening Newborns' Hearing Now Standard

    MedlinePlus

    ... this page please turn Javascript on. Feature: Taste, Smell, Hearing, Language, Voice, Balance Screening Newborns' Hearing Now ... emailing NIDCDinfo@nidcd.nih.gov . Read More "Taste, Smell, Hearing, Language, Voice, Balance" Articles At Last: A ...

  8. Age-Related Hearing Loss

    MedlinePlus

    ... hearing loss. Here are the most common ones: Styles of hearing aids Source: NIH/NIDCD Hearing aids ... list of organizations, contact: NIDCD Information Clearinghouse 1 Communication Avenue Bethesda, MD 20892-3456 Toll-free Voice: ( ...

  9. Hearing or speech impairment - resources

    MedlinePlus

    Resources - hearing or speech impairment ... The following organizations are good resources for information on hearing impairment or speech impairment: Alexander Graham Bell Association for the Deaf and Hard of Hearing -- www.agbell. ...

  10. Hearing protection for miners

    SciTech Connect

    Schulz, T.

    2008-10-15

    A NIOSH analysis showed that at age 50 approximately 90% of coal miners have a hearing impairment, yet noise included hearing loss is 100% preventable. The article discusses requirements of the MSHA regulations, 30 CFR Part 62 - occupational noise exposure (2000) and a 2008-MSHA document describing technologically achievable and promising controls for several types of mining machinery. Hearing protection is still required for exposure to greater than 90 dBA. These are now commercially available ways to determine how much attenuation an individual gets from a given hearing protector, known as 'fit testing'. 3 refs., 1 fig., 1 tab., 1 photo.

  11. [Implantable hearing aids].

    PubMed

    Luers, J C; Beutner, D; Hüttenbrink, K-B

    2011-10-01

    Strictly speaking, implantable hearing aids are technical systems that process audiological signals and convey these by direct mechanical stimulation of the ossicular chain or cochlea. They have certain benefits over conventional hearing aids in terms of wearing comfort and general acceptance. As current studies lack convincing audiological results, the indications for implantable hearing aids are primarily of medical or cosmetic nature. To date, three systems are available in Germany: Vibrant Soundbridge®, Carina®, and Esteem®. Because the performance of the different implantable and nonimplantable hearing systems together with various surgical procedures are currently undergoing major changes, audiological indications may also develop in the future. PMID:21956678

  12. SOX10 mutations mimic isolated hearing loss.

    PubMed

    Pingault, V; Faubert, E; Baral, V; Gherbi, S; Loundon, N; Couloigner, V; Denoyelle, F; Noël-Pétroff, N; Ducou Le Pointe, H; Elmaleh-Bergès, M; Bondurand, N; Marlin, S

    2015-10-01

    Ninety genes have been identified to date that are involved in non-syndromic hearing loss, and more than 300 different forms of syndromic hearing impairment have been described. Mutations in SOX10, one of the genes contributing to syndromic hearing loss, induce a large range of phenotypes, including several subtypes of Waardenburg syndrome and Kallmann syndrome with deafness. In addition, rare mutations have been identified in patients with isolated signs of these diseases. We used the recent characterization of temporal bone imaging aspects in patients with SOX10 mutations to identify possible patients with isolated hearing loss due to SOX10 mutation. We selected 21 patients with isolated deafness and temporal bone morphological defects for mutational screening. We identified two SOX10 mutations and found that both resulted in a non-functional protein in vitro. Re-evaluation of the two affected patients showed that both had previously undiagnosed olfactory defects. Diagnosis of anosmia or hyposmia in young children is challenging, and particularly in the absence of magnetic resonance imaging (MRI), SOX10 mutations can mimic non-syndromic hearing impairment. MRI should complete temporal bones computed tomographic scan in the management of congenital deafness as it can detect brain anomalies, cochlear nerve defects, and olfactory bulb malformation in addition to inner ear malformations. PMID:25256313

  13. Genetics of Hearing and Deafness

    PubMed Central

    ANGELI, SIMON; LIN, XI; LIU, XUE ZHONG

    2015-01-01

    This article is a review of the genes and genetic disorders that affect hearing in humans and a few selected mouse models of deafness. Genetics is playing an increasingly critical role in the practice of medicine. This is not only in part to the importance that genetic knowledge has on traditional genetic diseases but also in part to the fact that genetic knowledge provides an understanding of the fundamental biological process of most diseases. The proteins coded by the genes related to hearing loss (HL) are involved in many functions in the ear, such as cochlear fluid homeostasis, ionic channels, stereocilia morphology and function, synaptic transmission, gene regulation, and others. Mouse models play a crucial role in understanding of the pathogenesis associated with these genes. Different types of familial HL have been recognized for years; however, in the last two decades, there has been tremendous progress in the discovery of gene mutations that cause deafness. Most of the cases of genetic deafness recognized today are monogenic disorders that can be broadly classified by the mode of inheritance (i.e., autosomal dominant, autosomal recessive, X-linked, and mitochondrial inheritance) and by the presence of associated phenotypic features (i.e., syndromic; and nonsyndromic). In terms of nonsyndromic HL, the chromosomal locations are currently known for ~ 125 loci (54 for dominant and 71 for recessive deafness), 64 genes have been identified (24 for dominant and 40 for recessive deafness), and there are many more loci for syndromic deafness and X-linked and mitochondrial DNA disorders (http://hereditaryhearingloss.org). Thus, today’s clinician must understand the science of medical genetics as this knowledge can lead to more effective disease diagnosis, counseling, treatment, and prevention. PMID:23044516

  14. Hearing Loss and Cytomegalovirus.

    ERIC Educational Resources Information Center

    Strauss, Melvin

    1997-01-01

    Cytomegalovirus is the most common cause of congenital virally induced hearing loss. Maternal infection is most often asymptomatic as is the infection in the newborn. Hearing loss occurs in both clinically apparent infection and in the asymptomatic infection. Current methods of detection, treatment, and prevention and research efforts are…

  15. The Hearing Environment

    ERIC Educational Resources Information Center

    Capewell, Carmel

    2014-01-01

    Glue ear, a condition resulting in intermittent hearing loss in young children, affects about 80% of young children under seven years old. About 60% of children will spend a third of their time unable to hear within normal thresholds. Teachers are unlikely to consider the sound quality in classrooms. In my research young people provided…

  16. Hearing sensitivity in farmers.

    PubMed Central

    Karlovich, R S; Wiley, T L; Tweed, T; Jensen, D V

    1988-01-01

    Hearing sensitivity was measured for tones from 1,000 through 8,000 hertz (Hz) in 534 males and 278 females who resided in rural Wisconsin and ranged in age from 16 to 85 years. The hearing sensitivity for all subjects decreased with advancing age and at higher frequencies, but hearing loss over the range most susceptible to excessive noise exposure (3,000-6,000 Hz) was much greater for males than for females at all ages. The hearing loss was greater than could be accounted for by age and was similar whether the subject was a farmer or not. The results suggested that approximately 25 percent of the males had a communication handicap due to hearing loss by age 30, and the proportion rose to 50 percent by age 50. Less than 20 percent of farmers reported consistent use of personal hearing protection in their farm-related duties. Overall, the findings suggest that men who live in rural areas, including farmers, demonstrate a high prevalence of hearing loss and associated communication problems due to excessive noise exposure. This, in turn, clearly indicates a need for intensification of educational hearing conservation programs for the rural population. PMID:3124200

  17. [Presbycusis - Age Related Hearing Loss].

    PubMed

    Fischer, N; Weber, B; Riechelmann, H

    2016-07-01

    Presbycusis or age related hearing loss can be defined as a progressive, bilateral and symmetrical sensorineural hearing loss due to age related degeneration of inner ear structures. It can be considered a multifactorial complex disorder with environmental and genetic factors. The molecular, electrophysiological and histological damage at different levels of the inner ear cause a progressive hearing loss, which usually affects the high frequencies of hearing. The resulting poor speech recognition has a negative impact on cognitive, emotional and social function in older adults. Recent investigations revealed an association between hearing impairment and social isolation, anxiety, depression and cognitive decline in elderly. These findings emphasize the importance of diagnosis and treating hearing loss in the elderly population. Hearing aids are the most commonly used devices for treating presbycusis. The technical progress of implantable hearing devices allows an effective hearing rehabilitation even in elderly with severe hearing loss. However, most people with hearing impairments are not treated adequately. PMID:27392191

  18. Hearing impairment in Stickler syndrome: a systematic review

    PubMed Central

    2012-01-01

    Background Stickler syndrome is a connective tissue disorder characterized by ocular, skeletal, orofacial and auditory defects. It is caused by mutations in different collagen genes, namely COL2A1, COL11A1 and COL11A2 (autosomal dominant inheritance), and COL9A1 and COL9A2 (autosomal recessive inheritance). The auditory phenotype in Stickler syndrome is inconsistently reported. Therefore we performed a systematic review of the literature to give an up-to-date overview of hearing loss in Stickler syndrome, and correlated it with the genotype. Methods English-language literature was reviewed through searches of PubMed and Web of Science, in order to find relevant articles describing auditory features in Stickler patients, along with genotype. Prevalences of hearing loss are calculated and correlated with the different affected genes and type of mutation. Results 313 patients (102 families) individually described in 46 articles were included. Hearing loss was found in 62.9%, mostly mild to moderate when reported. Hearing impairment was predominantly sensorineural (67.8%). Conductive (14.1%) and mixed (18.1%) hearing loss was primarily found in young patients or patients with a palatal defect. Overall, mutations in COL11A1 (82.5%) and COL11A2 (94.1%) seem to be more frequently associated with hearing impairment than mutations in COL2A1 (52.2%). Conclusions Hearing impairment in patients with Stickler syndrome is common. Sensorineural hearing loss predominates, but also conductive hearing loss, especially in children and patients with a palatal defect, may occur. The distinct disease-causing collagen genes are associated with a different prevalence of hearing impairment, but still large phenotypic variation exists. Regular auditory follow-up is strongly advised, particularly because many Stickler patients are visually impaired. PMID:23110709

  19. Hearing Aid Tester

    NASA Technical Reports Server (NTRS)

    1978-01-01

    Hearing aids often develop malfunctions that are not detected by the wearer. This is particularly true when the wearers are school-age children. Studies of selected groups showed that from 30 to more than 50 percent of school children were not getting adequate benefit from their hearing aids because of unrecognized malfunctions, usually low or dead batteries. This can be serious because hearing impairment retards a child's educational progress. NASA technology incorporated in the Hearing Aid Malfunction Detection Unit (HAMDU), the device pictured, is expected to provide an effective countermeasure to the childrens' hearing aid problem. A patent license has been awarded to a minority-owned firm, Hopkins International Company, a subsidiary of H. H. Aerospace Design Co., Inc., Elmford, New York. The company plans early commercial availability of its version of the device.

  20. Sudden Sensorineural Hearing Loss

    PubMed Central

    Kuhn, Maggie; Heman-Ackah, Selena E.; Shaikh, Jamil A.

    2011-01-01

    Sudden sensorineural hearing loss (SSNHL) is commonly encountered in audiologic and otolaryngologic practice. SSNHL is most commonly defined as sensorineural hearing loss of 30dB or greater over at least three contiguous audiometric frequencies occurring within a 72-hr period. Although the differential for SSNHL is vast, for the majority of patients an etiologic factor is not identified. Treatment for SSNHL of known etiology is directed toward that agent, with poor hearing outcomes characteristic for discoverable etiologies that cause inner ear hair cell loss. Steroid therapy is the current mainstay of treatment of idiopathic SSNHL in the United States. The prognosis for hearing recovery for idiopathic SSNHL is dependent on a number of factors including the severity of hearing loss, age, presence of vertigo, and shape of the audiogram. PMID:21606048

  1. Individual Hearing Loss

    PubMed Central

    Dau, Torsten; Christensen-Dalsgaard, Jakob; Tranebjærg, Lisbeth; Andersen, Ture; Poulsen, Torben

    2016-01-01

    It is well-established that hearing loss does not only lead to a reduction of hearing sensitivity. Large individual differences are typically observed among listeners with hearing impairment in a wide range of suprathreshold auditory measures. In many cases, audiometric thresholds cannot fully account for such individual differences, which make it challenging to find adequate compensation strategies in hearing devices. How to characterize, model, and compensate for individual hearing loss were the main topics of the fifth International Symposium on Auditory and Audiological Research (ISAAR), held in Nyborg, Denmark, in August 2015. The following collection of papers results from some of the work that was presented and discussed at the symposium. PMID:27566802

  2. Mutational analysis of the mitochondrial 12S rRNA and tRNA{sup Ser(UCN)} genes in Tunisian patients with nonsyndromic hearing loss

    SciTech Connect

    Mkaouar-Rebai, Emna . E-mail: emna_mkaouar@mail2world.com; Tlili, Abdelaziz; Masmoudi, Saber; Louhichi, Nacim; Charfeddine, Ilhem; Amor, Mohamed Ben; Lahmar, Imed; Driss, Nabil; Drira, Mohamed; Ayadi, Hammadi; Fakhfakh, Faiza

    2006-02-24

    We explored the mitochondrial 12S rRNA and the tRNA{sup Ser(UCN)} genes in 100 Tunisian families affected with NSHL and in 100 control individuals. We identified the mitochondrial A1555G mutation in one out of these 100 families and not in the 100 control individuals. Members of this family harbouring the A1555G mutation showed phenotypic heterogeneity which could be explained by an eventual nuclear-mitochondrial interaction. So, we have screened three nuclear genes: GJB2, GJB3, and GJB6 but we have not found correlation between the phenotypic heterogeneity and variants detected in these genes. We explored also the entire mitochondrial 12S rRNA and the tRNA{sup Ser(UCN)} genes. We detected five novel polymorphisms: T742C, T794A, A813G, C868T, and C954T, and 12 known polymorphisms in the mitochondrial 12S rRNA gene. None of the 100 families or the 100 controls were found to carry mutations in the tRNA{sup Ser(UCN)} gene. We report here First mutational screening of the mitochondrial 12S rRNA and the tRNA{sup Ser(UCN)} genes in the Tunisian population which describes the second family harbouring the A1555G mutation in Africa and reveals novel polymorphisms in the mitochondrial 12S rRNA gene.

  3. Hearing Aids and Hearing Impaired Students in Rural Schools.

    ERIC Educational Resources Information Center

    Woodford, Charles

    This paper describes functions of the components of hearing aids and provides a detailed procedure to detect hearing aid dysfunctions. The most common type of hearing aids for school children are the behind the ear type. Various hearing aid components change sound into an electrical signal, which is amplified and adjusted by a volume control. The…

  4. Syntactic Performance of Hearing Impaired and Normal Hearing Individuals.

    ERIC Educational Resources Information Center

    Quigley, Stephen P.; King, Cynthia M.

    1980-01-01

    Describes two research programs on the syntactic abilities of hearing impaired and normal hearing individuals. The first program involved deaf students acquiring English structure; the second involved the construction of the Test of Syntactic Abilities and its application to deaf, hard of hearing, and normal hearing students in the United States,…

  5. Hearing Impairment and Retirement

    PubMed Central

    Fischer, Mary E; Cruickshanks, Karen J; Pinto, Alex; Klein, Barbara E K; Klein, Ronald; Dalton, Dayna S

    2013-01-01

    BACKGROUND Many factors influence the decision to retire including age, insurance and pension availability along with physical and mental health. Hearing impairment may be one such factor. PURPOSE The purpose of this study was to compare the 15 year retirement rate among subjects with and without hearing impairment. RESEARCH DESIGN Prospective, population-based study STUDY SAMPLE Subjects were participants in the Epidemiology of Hearing Loss Study (EHLS), a longitudinal investigation of age-related hearing loss. Participants who were working full- or part-time in 1993–1995 were included (n=1410, mean age=57.8 years). DATA COLLECTION AND ANALYSIS Data from four EHLS phases (1993–1995, 1998–2000, 2003–2005, and 2009–2010) were analyzed in 2010–2012. Hearing impairment was defined as a pure tone threshold average (at 0.5,1,2 and 4 kHz) greater than 25 dB HL in the worse ear. Employment status was determined at each of the four phases. Kaplan-Meier estimates of the cumulative incidence of retirement were calculated and Cox discrete-time modeling was used to determine the effect of hearing impairment on the rate of retirement. RESULTS The cumulative incidence of retirement was significantly (p < 0.02) higher in those with a hearing impairment (77%) compared to those without a hearing impairment (74%). After adjustment for age, gender, self-reported health, and history of chronic disease, there was no significant difference in the rate of retirement between those with and without a hearing impairment (Hazard Ratio (HR) = 0.9, 95% Confidence Interval (CI) = 0.7, 1.1). Similar results were observed when hearing aid users were excluded, when hearing impairment was based on the better ear thresholds, and when analyses were restricted to those less than 65 years of age and working full-time at baseline. Participants with a hearing impairment were less likely to state that the main reason for retirement was that the time seemed right. CONCLUSIONS Hearing impairment

  6. Noise and Hearing Loss Prevention

    MedlinePlus

    ... SafeInSound Noise and Hearing Loss on the NIOSH Science Blog Smartphone Sound Apps Music-induced Hearing Loss ... SafeInSound Noise and Hearing Loss on the NIOSH Science Blog Smartphone Sound Apps Music-induced Hearing Loss ...

  7. Can You Hear Me Now?

    ERIC Educational Resources Information Center

    Black, Susan

    2003-01-01

    Almost 15 percent of children from 6 to 19 years old have some degree of hearing loss in one or both ears. Offers guidelines for screening students' hearing and recommends that interventions begin with classroom acoustics, which affect all children including those with normal hearing. Includes strategies for accommodating hearing-impaired…

  8. Pseudo-immortalization of postnatal cochlear progenitor cells yields a scalable cell line capable of transcriptionally regulating mature hair cell genes

    PubMed Central

    Walters, Brandon J.; Diao, Shiyong; Zheng, Fei; Walters, Bradley J.; Layman, Wanda S.; Zuo, Jian

    2015-01-01

    The mammalian cochlea is a highly specialized organ within the inner ear. Sensory hair cells (HC) in the cochlea detect and transduce sound waves into electrical impulses that are sent to the brain. Studies of the molecular pathways regulating HC formation are hindered by the very sparse nature of HCs, where only ~3300 are found within an entire mouse cochlea. Current cell lines mimic certain aspects of HCs but lack terminal HC marker expression. Here we successfully “pseudo-immortalized” cochlear progenitor cells using the “conditional reprogramming” technique. These cells, termed “Conditionally Reprogrammed Otic Stem Cells” (CR-OSC), are able to bypass the senescence inherent to cochlear progenitor cells without genetic alterations, allowing for the generation of over 15 million cells from a single cochlea. These cells can be differentiated and up-regulate both early and terminal differentiation genes associated with HCs, including the terminal HC differentiation marker prestin. CR-OSCs also respond to known HC cues, including upregulation of HC genes in response to Atoh1 overexpression, and upregulation of prestin expression after thyroid hormone application. Overall, we describe the creation of a HC line capable of regulated expression of HC genes that can easily be recreated in any laboratory from any mouse of interest. PMID:26639154

  9. Pseudo-immortalization of postnatal cochlear progenitor cells yields a scalable cell line capable of transcriptionally regulating mature hair cell genes.

    PubMed

    Walters, Brandon J; Diao, Shiyong; Zheng, Fei; Walters, Bradley J; Layman, Wanda S; Zuo, Jian

    2015-01-01

    The mammalian cochlea is a highly specialized organ within the inner ear. Sensory hair cells (HC) in the cochlea detect and transduce sound waves into electrical impulses that are sent to the brain. Studies of the molecular pathways regulating HC formation are hindered by the very sparse nature of HCs, where only ~3300 are found within an entire mouse cochlea. Current cell lines mimic certain aspects of HCs but lack terminal HC marker expression. Here we successfully "pseudo-immortalized" cochlear progenitor cells using the "conditional reprogramming" technique. These cells, termed "Conditionally Reprogrammed Otic Stem Cells" (CR-OSC), are able to bypass the senescence inherent to cochlear progenitor cells without genetic alterations, allowing for the generation of over 15 million cells from a single cochlea. These cells can be differentiated and up-regulate both early and terminal differentiation genes associated with HCs, including the terminal HC differentiation marker prestin. CR-OSCs also respond to known HC cues, including upregulation of HC genes in response to Atoh1 overexpression, and upregulation of prestin expression after thyroid hormone application. Overall, we describe the creation of a HC line capable of regulated expression of HC genes that can easily be recreated in any laboratory from any mouse of interest. PMID:26639154

  10. Hearing in Cavefishes.

    PubMed

    Soares, Daphne; Niemiller, Matthew L; Higgs, Dennis M

    2016-01-01

    Caves and associated subterranean habitats represent some of the harshest environments on Earth, yet many organisms, including fishes, have colonized and thrive in these habitats despite the complete absence of light, and other abiotic and biotic constraints. Over 170 species of fishes are considered obligate subterranean inhabitants (stygobionts) that exhibit some degree of troglomorphy, including degeneration of eyes and reduction in pigmentation. To compensate for lack of vision, many species have evolved constructive changes to non-visual sensory modalities. In this chapter we review hearing in cavefishes, with particular emphasize on our own studies on amblyopsid cavefishes. Hearing in cavefishes has not been well studied to date, as hearing ability has only been examined in four species. Two species show no differences in hearing ability relative to their surface relatives, while the other two species (family Amblyopsidae) exhibit regression in the form of reduced hearing range and reduction in hair cell densities on sensory epithelia. In addition to reviewing our current knowledge on cavefish hearing, we offer suggestions for future avenues of research on cavefish hearing and discuss the influence of Popper and Fay on the field of cavefish bioacoustics. PMID:26515315

  11. Micromechanics of hearing

    NASA Astrophysics Data System (ADS)

    Hudspeth, A. J.

    2015-12-01

    The following summarizes the key points addressed during a tutorial session on the Micromechanics of Hearing that took place at the 12th International Workshop on the Mechanics of Hearing held at Cape Sounio, Greece, in June 2014. The tutorial was intended to present an overview of basic ideas and to address topics of current interest relevant to the Workshop. The session was recorded, and the audio file and accompanying visual content of the presentation can be found in the Mechanics of Hearing Digital Library (www.mechanicsofhearing.org).

  12. Upcoming hearings in Congress

    NASA Astrophysics Data System (ADS)

    The following hearings have been tentatively scheduled for the coming weeks by the Senate and House of Representatives. Dates and times should be verified with the committee or subcommittee holding the hearing; all congressional offices may be reached by telephoning 202-224-3121. For guidelines on contacting a member of Congress, see AGU's Guide to Legislative Information and Contacts (Eos, August 28, 1984, p. 669).April 25: Oversight hearing on submerged lands by the Public Lands Subcommittee of the House Interior and Insular Affairs Committee. Room to be assigned, 9:45 A.M.

  13. The application of genome editing in studying hearing loss.

    PubMed

    Zou, Bing; Mittal, Rahul; Grati, M'hamed; Lu, Zhongmin; Shu, Yilai; Tao, Yong; Feng, Youg; Xie, Dinghua; Kong, Weijia; Yang, Shiming; Chen, Zheng-Yi; Liu, Xuezhong

    2015-09-01

    Targeted genome editing mediated by clustered, regularly interspaced, short palindromic repeat (CRISPR)/CRISPR-associated nuclease 9 (Cas9) technology has emerged as one of the most powerful tools to study gene functions, and with potential to treat genetic disorders. Hearing loss is one of the most common sensory disorders, affecting approximately 1 in 500 newborns with no treatment. Mutations of inner ear genes contribute to the largest portion of genetic deafness. The simplicity and robustness of CRISPR/Cas9-directed genome editing in human cells and model organisms such as zebrafish, mice and primates make it a promising technology in hearing research. With CRISPR/Cas9 technology, functions of inner ear genes can be studied efficiently by the disruption of normal gene alleles through non-homologous-end-joining (NHEJ) mechanism. For genetic hearing loss, CRISPR/Cas9 has potential to repair gene mutations by homology-directed-repair (HDR) or to disrupt dominant mutations by NHEJ, which could restore hearing. Our recent work has shown CRISPR/Cas9-mediated genome editing can be efficiently performed in the mammalian inner ear in vivo. Thus, application of CRISPR/Cas9 in hearing research will open up new avenues for understanding the pathology of genetic hearing loss and provide new routes in the development of treatment to restore hearing. In this review, we describe major methodologies currently used for genome editing. We will highlight applications of these technologies in studies of genetic disorders and discuss issues pertaining to applications of CRISPR/Cas9 in auditory systems implicated in genetic hearing loss. PMID:25987504

  14. Screening of Connexin 26 in Nonsyndromic Hearing Loss

    PubMed Central

    Moreira, Danielle; Silva, Daniela da; Lopez, Priscila; Mantovani, Jair Cortez

    2014-01-01

    Introduction The first locus for nonsyndromic autosomal recessive hearing loss is on chromosome 13q11–22. The 35delG mutation is present in 80% of cases in which GJB2 is involved, which makes the study of this mutation very important. The viability and benefits of screening for mutations in the connexin 26 gene are now beginning to change the diagnostic evaluation and identification of the etiology of hearing loss. Objective To investigate the occurrence of the 35delG mutation in patients with nonsyndromic sensorineural hearing loss and their first degree relatives. Methods This transversal study included 72 patients from the local hospital. The patients were divided into three groups: group A, sensorineural hearing loss (n = 58); group B, first-degree relatives of group A with sensorineural hearing loss (n = 09); and group C, first-degree relatives of patients from group A without hearing loss (n = 05). All patients had audiological evaluation and genetic testing of the 35delG mutation. Results The 35delG mutation was found in four heterozygous mutations (three of them found in the same family). The other heterozygous mutation was found in a female patient with bilateral, moderate, prelingual, sensorineural hearing loss. A single homozygous mutation was found in a male patient, with severe sensorineural hearing loss in his right ear and profound hearing loss in the left ear. Conclusions The 35delG mutation was found in 7% of the cases. The test is easy to perform and inexpensive, but it is necessary to investigate other genes related to hearing loss. PMID:25992148

  15. Can Baby Hear?

    MedlinePlus

    ... Current Issue Past Issues Special Section: Focus on Communication Can Baby Hear? Past Issues / Fall 2008 Table ... to the National Institute on Deafness and Other Communication Disorders (NIDCD). Prior to this, the average age ...

  16. Devices for hearing loss

    MedlinePlus

    ... bring the sound from your TV, radio, or music player directly to your inner ear. Many listening devices now work through a wireless link and can connect directly to your hearing aid. There is also television closed-captioning, which ...

  17. Implantable digital hearing aid

    NASA Technical Reports Server (NTRS)

    Kissiah, A. M., Jr.

    1979-01-01

    Hearing aid converts analog output of microphone into digital pulses in about 10 channels of audiofrequencies. Each pulse band could be directly connected to portion of auditory nerve most sensitive to that range.

  18. Types of Hearing Aids

    MedlinePlus

    ... They also have greater flexibility in hearing aid programming so that the sound they transmit can be ... 10903 New Hampshire Avenue Silver Spring, MD 20993 1-888-INFO-FDA (1-888-463-6332) Contact ...

  19. Living with Hearing Loss

    MedlinePlus

    ... Current Issue Past Issues Special Section: Focus on Communication Living with Hearing Loss Past Issues / Fall 2008 ... the United States suffer some form of disordered communication. The National Institute on Deafness and Other Communication ...

  20. Occupational hearing loss

    MedlinePlus

    Over time, repeated exposure to loud noise and music can cause hearing loss. Sounds above 80 decibels ( ... Airline ground maintenance Construction Farming Jobs involving loud music or machinery In the U.S., laws regulate the ...

  1. Occupational hearing loss

    MedlinePlus

    ... risks connected with recreation such as shooting a gun, driving snowmobiles, or other, similar activities. Learn how ... hearing from recreational activities such as shooting a gun or driving snowmobiles. Do not listen to loud ...

  2. Hearing loss - infants

    MedlinePlus

    ... to sounds through play. These tests, known as visual response audiometry and play audiometry, can better determine ... the cause of hearing loss. Treatment may include: Speech therapy Learning sign language Cochlear implant (for those ...

  3. Hearing loss and music

    MedlinePlus

    ... iPod or MP3 Player The small ear bud style headphones (inserted into the ears) do not block ... Hearing Loss. National Institute on Deafness and Other Communication Disorders. NIH Pub. No. 14-4233. Updated: March ...

  4. Genomic sequencing and analyses of HearMNPV—a new Multinucleocapsid nucleopolyhedrovirus isolated from Helicoverpa armigera

    PubMed Central

    2012-01-01

    Background HearMNPV, a nucleopolyhedrovirus (NPV), which infects the cotton bollworm, Helicoverpa armigera, comprises multiple rod-shaped nucleocapsids in virion(as detected by electron microscopy). HearMNPV shows a different host range compared with H. armigera single-nucleocapsid NPV (HearSNPV). To better understand HearMNPV, the HearMNPV genome was sequenced and analyzed. Methods The morphology of HearMNPV was observed by electron microscope. The qPCR was used to determine the replication kinetics of HearMNPV infectious for H. armigera in vivo. A random genomic library of HearMNPV was constructed according to the “partial filling-in” method, the sequence and organization of the HearMNPV genome was analyzed and compared with sequence data from other baculoviruses. Results Real time qPCR showed that HearMNPV DNA replication included a decreasing phase, latent phase, exponential phase, and a stationary phase during infection of H. armigera. The HearMNPV genome consists of 154,196 base pairs, with a G + C content of 40.07%. 162 putative ORFs were detected in the HearMNPV genome, which represented 90.16% of the genome. The remaining 9.84% constitute four homologous regions and other non-coding regions. The gene content and gene arrangement in HearMNPV were most similar to those of Mamestra configurata NPV-B (MacoNPV-B), but was different to HearSNPV. Comparison of the genome of HearMNPV and MacoNPV-B suggested that HearMNPV has a deletion of a 5.4-kb fragment containing five ORFs. In addition, HearMNPV orf66, bro genes, and hrs are different to the corresponding parts of the MacoNPV-B genome. Conclusions HearMNPV can replicate in vivo in H. armigera and in vitro, and is a new NPV isolate distinguished from HearSNPV. HearMNPV is most closely related to MacoNPV-B, but has a distinct genomic structure, content, and organization. PMID:22913743

  5. Diagnosis of Hearing Loss.

    ERIC Educational Resources Information Center

    World Federation of the Deaf, Rome (Italy).

    Seven conference papers from the U.S.S.R., India, Poland, Czechoslovakia, and Yugoslavia consider the diagnosis of hearing loss. They are "Examination of Hearing of Children, Aged from 2 to 5, by Means of Playing Audiometry" by A. P. Kossacheva, "A Study of the Etiology and Pattern of Deafness in a School for the Deaf in Madras, South India" by Y.…

  6. Hearing Aids and Music

    PubMed Central

    Chasin, Marshall; Russo, Frank A.

    2004-01-01

    Historically, the primary concern for hearing aid design and fitting is optimization for speech inputs. However, increasingly other types of inputs are being investigated and this is certainly the case for music. Whether the hearing aid wearer is a musician or merely someone who likes to listen to music, the electronic and electro-acoustic parameters described can be optimized for music as well as for speech. That is, a hearing aid optimally set for music can be optimally set for speech, even though the converse is not necessarily true. Similarities and differences between speech and music as inputs to a hearing aid are described. Many of these lead to the specification of a set of optimal electro-acoustic characteristics. Parameters such as the peak input-limiting level, compression issues—both compression ratio and knee-points—and number of channels all can deleteriously affect music perception through hearing aids. In other cases, it is not clear how to set other parameters such as noise reduction and feedback control mechanisms. Regardless of the existence of a “music program,” unless the various electro-acoustic parameters are available in a hearing aid, music fidelity will almost always be less than optimal. There are many unanswered questions and hypotheses in this area. Future research by engineers, researchers, clinicians, and musicians will aid in the clarification of these questions and their ultimate solutions. PMID:15497032

  7. [Progressive hearing loss].

    PubMed

    Reiss, M; Reiss, G

    2000-01-01

    Progressive sensorineural hearing loss (SNHL) is defined as hearing loss of unknown etiology with fairly high-speed progression. Its diagnostic criteria consist of the following: that it is 1) progressive, 2) with bilateral involvement, and 3) of unknown etiology. Due to recent advances in diagnostics, imaging and management, SNHL has gained much interest from otologists in the last few years. They provide new insight into the physiology and pathophysiology of hearing. SNHL which is sudden in onset, fluctuating, and/or progressive complicates medical management, hearing aid selection, and individualized educational planning for a hearing-impaired patient. Existing hypotheses on the etiology of SNHL are judged on experimental, clinical, laboratory and radiological evidence. Cardiovascular and rheologic diseases, hereditary disorders, immunological phenomena, infections, environmental causes like noise, ototoxic drugs and industrial substances and systemic maladies must be included in the diagnostic reflections. Potential concepts of treatment include rheologic medications and corticosteroids. Hearing aids and timely cochlear implant operation are further possible forms of treatment. PMID:10893764

  8. 41 CFR 105-57.005 - Hearing.

    Code of Federal Regulations, 2011 CFR

    2011-01-01

    ...-ADMINISTRATION WAGE GARNISHMENT § 105-57.005 Hearing. (a) GSA will provide a hearing, which at the hearing... summary of the facts presented; (2) The hearing official's findings, analysis and conclusions; and (3)...

  9. The MYC Road to Hearing Restoration

    PubMed Central

    Kopecky, Benjamin; Fritzsch, Bernd

    2012-01-01

    Current treatments for hearing loss, the most common neurosensory disorder, do not restore perfect hearing. Regeneration of lost organ of Corti hair cells through forced cell cycle re-entry of supporting cells or through manipulation of stem cells, both avenues towards a permanent cure, require a more complete understanding of normal inner ear development, specifically the balance of proliferation and differentiation required to form and to maintain hair cells. Direct successful alterations to the cell cycle result in cell death whereas regulation of upstream genes is insufficient to permanently alter cell cycle dynamics. The Myc gene family is uniquely situated to synergize upstream pathways into downstream cell cycle control. There are three Mycs that are embedded within the Myc/Max/Mad network to regulate proliferation. The function of the two ear expressed Mycs, N-Myc and L-Myc were unknown less than two years ago and their therapeutic potentials remain speculative. In this review, we discuss the roles the Mycs play in the body and what led us to choose them to be our candidate gene for inner ear therapies. We will summarize the recently published work describing the early and late effects of N-Myc and L-Myc on hair cell formation and maintenance. Lastly, we detail the translational significance of our findings and what future work must be performed to make the ultimate hearing aid: the regeneration of the organ of Corti. PMID:24710525

  10. 18 CFR 401.84 - Hearing procedure.

    Code of Federal Regulations, 2010 CFR

    2010-04-01

    ... ADMINISTRATIVE MANUAL RULES OF PRACTICE AND PROCEDURE Administrative and Other Hearings § 401.84 Hearing procedure. (a) Participation in the hearing. In any hearing, the person requesting the hearing shall be... 18 Conservation of Power and Water Resources 2 2010-04-01 2010-04-01 false Hearing procedure....

  11. 18 CFR 1308.33 - Hearings.

    Code of Federal Regulations, 2011 CFR

    2011-04-01

    ... 18 Conservation of Power and Water Resources 2 2011-04-01 2011-04-01 false Hearings. 1308.33... Prehearing and Hearing Procedures § 1308.33 Hearings. (a) TVA shall arrange for the verbatim reporting of evidentiary hearings before the Hearing Officer, and shall provide the Hearing Officer with the...

  12. 18 CFR 1308.33 - Hearings.

    Code of Federal Regulations, 2010 CFR

    2010-04-01

    ... 18 Conservation of Power and Water Resources 2 2010-04-01 2010-04-01 false Hearings. 1308.33... Prehearing and Hearing Procedures § 1308.33 Hearings. (a) TVA shall arrange for the verbatim reporting of evidentiary hearings before the Hearing Officer, and shall provide the Hearing Officer with the...

  13. 20 CFR 658.417 - Hearings.

    Code of Federal Regulations, 2010 CFR

    2010-04-01

    ... 20 Employees' Benefits 3 2010-04-01 2010-04-01 false Hearings. 658.417 Section 658.417 Employees... Hearings. (a) Hearings shall be held by State hearing officials. A State hearing official may be any State official authorized to hold hearings under State law. They may be, for example, the same referees who...

  14. 42 CFR 431.222 - Group hearings.

    Code of Federal Regulations, 2010 CFR

    2010-10-01

    ... 42 Public Health 4 2010-10-01 2010-10-01 false Group hearings. 431.222 Section 431.222 Public... Recipients Right to Hearing § 431.222 Group hearings. The agency— (a) May respond to a series of individual requests for hearing by conducting a single group hearing; (b) May consolidate hearings only in cases...

  15. 42 CFR 431.222 - Group hearings.

    Code of Federal Regulations, 2011 CFR

    2011-10-01

    ... 42 Public Health 4 2011-10-01 2011-10-01 false Group hearings. 431.222 Section 431.222 Public... Recipients Right to Hearing § 431.222 Group hearings. The agency— (a) May respond to a series of individual requests for hearing by conducting a single group hearing; (b) May consolidate hearings only in cases...

  16. 20 CFR 410.645 - Joint hearings.

    Code of Federal Regulations, 2011 CFR

    2011-04-01

    ... 20 Employees' Benefits 2 2011-04-01 2011-04-01 false Joint hearings. 410.645 Section 410.645..., Finality of Decisions, and Representation of Parties § 410.645 Joint hearings. When two or more hearings... joint hearing, a joint hearing may not be held. Where joint hearings are held, a single record of...

  17. 20 CFR 410.645 - Joint hearings.

    Code of Federal Regulations, 2010 CFR

    2010-04-01

    ... 20 Employees' Benefits 2 2010-04-01 2010-04-01 false Joint hearings. 410.645 Section 410.645..., Finality of Decisions, and Representation of Parties § 410.645 Joint hearings. When two or more hearings... joint hearing, a joint hearing may not be held. Where joint hearings are held, a single record of...

  18. Hearing is Believing

    NASA Technical Reports Server (NTRS)

    2003-01-01

    This paper presents a discussion on the cochlear implant. This device was developed by Adam Kissiah, who suffers from hearing loss. Driven by his own hearing problem and three failed corrective surgeries, Kissiah started working in the mid-1970s on this surgically implantable device that provides hearing sensation to persons with severe-to-profound hearing loss who receive little or no benefit from hearing aids. Uniquely, the cochlear implant concept was not based on theories of medicine, as Kissiah had no medical background whatsoever. Instead, he utilized the technical expertise he learned while working as an electronics instrumentation engineer at NASA s Kennedy Space Center for the basis of his invention. This took place over 3 years, when Kissiah would spend his lunch breaks and evenings in Kennedy s technical library, studying the impact of engineering principles on the inner ear. In April of 2003, Kissiah was inducted into the Space Foundation's U.S. Space Technology Hall of Fame for his invention

  19. Underwater Hearing in Turtles.

    PubMed

    Willis, Katie L

    2016-01-01

    The hearing of turtles is poorly understood compared with the other reptiles. Although the mechanism of transduction of sound into a neural signal via hair cells has been described in detail, the rest of the auditory system is largely a black box. What is known is that turtles have higher hearing thresholds than other reptiles, with best frequencies around 500 Hz. They also have lower underwater hearing thresholds than those in air, owing to resonance of the middle ear cavity. Further studies demonstrated that all families of turtles and tortoises share a common middle ear cavity morphology, with scaling best suited to underwater hearing. This supports an aquatic origin of the group. Because turtles hear best under water, it is important to examine their vulnerability to anthropogenic noise. However, the lack of basic data makes such experiments difficult because only a few species of turtles have published audiograms. There are also almost no behavioral data available (understandable due to training difficulties). Finally, few studies show what kinds of sounds are behaviorally relevant. One notable paper revealed that the Australian snake-necked turtle (Chelodina oblonga) has a vocal repertoire in air, at the interface, and under water. Findings like these suggest that there is more to the turtle aquatic auditory scene than previously thought. PMID:26611091

  20. Hearing speech in music.

    PubMed

    Ekström, Seth-Reino; Borg, Erik

    2011-01-01

    The masking effect of a piano composition, played at different speeds and in different octaves, on speech-perception thresholds was investigated in 15 normal-hearing and 14 moderately-hearing-impaired subjects. Running speech (just follow conversation, JFC) testing and use of hearing aids increased the everyday validity of the findings. A comparison was made with standard audiometric noises [International Collegium of Rehabilitative Audiology (ICRA) noise and speech spectrum-filtered noise (SPN)]. All masking sounds, music or noise, were presented at the same equivalent sound level (50 dBA). The results showed a significant effect of piano performance speed and octave (P<.01). Low octave and fast tempo had the largest effect; and high octave and slow tempo, the smallest. Music had a lower masking effect than did ICRA noise with two or six speakers at normal vocal effort (P<.01) and SPN (P<.05). Subjects with hearing loss had higher masked thresholds than the normal-hearing subjects (P<.01), but there were smaller differences between masking conditions (P<.01). It is pointed out that music offers an interesting opportunity for studying masking under realistic conditions, where spectral and temporal features can be varied independently. The results have implications for composing music with vocal parts, designing acoustic environments and creating a balance between speech perception and privacy in social settings. PMID:21768731

  1. 42 CFR 405.720 - Hearing; right to hearing.

    Code of Federal Regulations, 2010 CFR

    2010-10-01

    ... 42 Public Health 2 2010-10-01 2010-10-01 false Hearing; right to hearing. 405.720 Section 405.720 Public Health CENTERS FOR MEDICARE & MEDICAID SERVICES, DEPARTMENT OF HEALTH AND HUMAN SERVICES MEDICARE PROGRAM FEDERAL HEALTH INSURANCE FOR THE AGED AND DISABLED Reconsiderations and Appeals Under Medicare Part A § 405.720 Hearing; right to...

  2. 42 CFR 405.720 - Hearing; right to hearing.

    Code of Federal Regulations, 2011 CFR

    2011-10-01

    ... 42 Public Health 2 2011-10-01 2011-10-01 false Hearing; right to hearing. 405.720 Section 405.720 Public Health CENTERS FOR MEDICARE & MEDICAID SERVICES, DEPARTMENT OF HEALTH AND HUMAN SERVICES MEDICARE PROGRAM FEDERAL HEALTH INSURANCE FOR THE AGED AND DISABLED Reconsiderations and Appeals Under Medicare Part A § 405.720 Hearing; right to...

  3. 49 CFR 1113.1 - Scheduling hearings; continued hearings.

    Code of Federal Regulations, 2010 CFR

    2010-10-01

    ... 49 Transportation 8 2010-10-01 2010-10-01 false Scheduling hearings; continued hearings. 1113.1 Section 1113.1 Transportation Other Regulations Relating to Transportation (Continued) SURFACE TRANSPORTATION BOARD, DEPARTMENT OF TRANSPORTATION RULES OF PRACTICE ORAL HEARING § 1113.1 Scheduling...

  4. How to Get Hearing Aids

    MedlinePlus

    ... to determine the type and amount of your hearing loss. The process begins with a medical and audiologic ... to rule out any medical reason for your hearing loss, such as infection, injury or deformity, ear wax ...

  5. 78 FR 21632 - Investigative Hearing

    Federal Register 2010, 2011, 2012, 2013, 2014

    2013-04-11

    ... hearing will be to gather additional information on the selection of the lithium ion (Li- ion) battery... compliance for the Boeing 787 Li-ion battery system. Parties to the hearing include the Federal...

  6. Hearing, Ear Infections, and Deafness

    MedlinePlus

    ... Involved You are here Home » Health Info Hearing, Ear Infections, and Deafness Diseases and Conditions Age-Related ... Do You Need a Hearing Test? Interactive Quiz Ear Infections in Children Enlarged Vestibular Aqueducts and Childhood ...

  7. 77 FR 44703 - Public Hearing

    Federal Register 2010, 2011, 2012, 2013, 2014

    2012-07-30

    ... From the Federal Register Online via the Government Publishing Office SUSQUEHANNA RIVER BASIN COMMISSION Public Hearing AGENCY: Susquehanna River Basin Commission. ACTION: Notice. SUMMARY: The Susquehanna River Basin Commission will hold a public hearing on August 23, 2012, in Harrisburg,...

  8. 77 FR 64576 - Public Hearing

    Federal Register 2010, 2011, 2012, 2013, 2014

    2012-10-22

    ... From the Federal Register Online via the Government Publishing Office SUSQUEHANNA RIVER BASIN COMMISSION Public Hearing AGENCY: Susquehanna River Basin Commission. ACTION: Notice. SUMMARY: The Susquehanna River Basin Commission will hold a public hearing on November 15, 2012, in...

  9. 78 FR 5556 - Public Hearing

    Federal Register 2010, 2011, 2012, 2013, 2014

    2013-01-25

    ... From the Federal Register Online via the Government Publishing Office SUSQUEHANNA RIVER BASIN COMMISSION Public Hearing AGENCY: Susquehanna River Basin Commission. ACTION: Notice. SUMMARY: The Susquehanna River Basin Commission will hold a public hearing on February 14, 2013, in...

  10. [Hearing disorders and rock music].

    PubMed

    Lindhardt, Bjarne Orskov

    2008-12-15

    Only few studies have investigated the frequency of hearing disorders in rock musicians. Performing rock music is apparently associated with a hearing loss in a fraction of musicians. Tinnitus and hyperacusis are more common among rock musicians than among the background population. It seems as if some sort of resistance against further hearing loss is developed over time. The use of ear protection devices have not been studied systematically but appears to be associated with diminished hearing loss. PMID:19128557

  11. Congenital sensorineural hearing loss

    SciTech Connect

    Mafee, M.F.; Selis, J.E.; Yannias, D.A.; Valvassori, G.E.; Pruzansky, S.; Applebaum, E.L.; Capek, V.

    1984-02-01

    The ears of 47 selected patients with congenital sensorineural hearing loss were examined with complex-motion tomography. The patients were divided into 3 general categories: those with a recognized syndrome, those with sensorineural hearing loss unrelated to any known syndrome, and those with microtia. A great variety of inner ear anomalies was detected, but rarely were these characteristic of a particular clinical entity. The most common finding was the Mondini malformation or one of its variants. Isolated dysplasia of the internal auditory canal or the vestibular aqueduct may be responsible for sensorineural hearing loss in some patients. Patients with microtia may also have severe inner ear abnormalities despite the fact that the outer and inner ears develop embryologically from completely separate systems.

  12. Upcoming hearings in Congress

    NASA Astrophysics Data System (ADS)

    The following hearings and markups have been tentatively scheduled for the coming weeks by the Senate and House of Representatives. Dates and times should be verified with the committee or subcommittee holding the hearing or markup; all offices on Capitol Hill may be reached by telephoning 202-224-3121. For guidelines on contacting a member of Congress, see AGU's Guide to Legislative Information and Contacts (Eos, August 28, 1984, p. 669).October 8: A joint hearing by the Energy Research & Development Subcommittee of the Senate Energy and Natural Resources Committee and the Nuclear Regulation Subcommittee of the Senate Environment and Public Works Committee on low-level radioactive waste (S. 1517 and S. 1578). Room SD-366, Dirksen Building, 9:30 A.M.

  13. 78 FR 11237 - Public Hearing

    Federal Register 2010, 2011, 2012, 2013, 2014

    2013-02-15

    ... SAFETY BOARD Public Hearing On Tuesday, February 26, 2013 the National Transportation Safety Board (NTSB) will convene an Investigative Hearing to gather additional factual information for the ongoing...) intermodal train No. AAMMLX-22 on June 24, 2012 near Goodwell, Oklahoma. The hearing will begin at 9:00...

  14. Eldercare at Home: Hearing Problems

    MedlinePlus

    ... members and friends to comment on how much communication has improved when your mother is wearing the hearing aid. If your mother is concerned about the appearance of a hearing aid, tell her that most women can style their hair to cover the hearing aids. Problem " ...

  15. HEARING IMPAIRMENT IN CHILDREN

    PubMed Central

    Kohlmoos, H. W.

    1953-01-01

    Abnormal behavior in children may frequently be caused by impairment of hearing. Early detection of the impairment and of the cause are of utmost importance, not only to prevent irreversible changes where that is possible, but to permit early beginning of special training for children who are permanently deaf. Recent studies have shown that deafness of infants may follow rubella in the mother in early pregnancy, or kernicterus caused by Rh incompatibilities. Measures to control these disorders are being investigated. Adequate and careful treatment of diseases of the nose, as well as surgical drainage of infected ears when necessary, are important factors in the prevention of hearing loss in children. PMID:13009516

  16. 43 CFR 4.1364 - Time for hearing; notice of hearing; extension of time for hearing.

    Code of Federal Regulations, 2010 CFR

    2010-10-01

    ... 43 Public Lands: Interior 1 2010-10-01 2010-10-01 false Time for hearing; notice of hearing; extension of time for hearing. 4.1364 Section 4.1364 Public Lands: Interior Office of the Secretary of the... Revisions, Permit Renewals, the Transfer, Assignment Or Sale of Rights Granted Under Permit (federal...

  17. Autism and Hearing Loss.

    ERIC Educational Resources Information Center

    Rosenhall, Ulf; Nordin, Viviann; Sandstrom, Mikael; Ahlsen, Gunilla; Gillberg, Christopher

    1999-01-01

    Children and adolescents (N=199) with autistic disorder were audiologically evaluated. Mild to moderate hearing loss was diagnosed in 7.9 percent, with deafness diagnosed in 3.5 percent of all cases, which represented a prevalence considerably above that in the general population and comparable to the prevalence found in populations with mental…

  18. Hear and Now

    ERIC Educational Resources Information Center

    McKeon, Michael; Berry, Lincoln

    2006-01-01

    Classrooms often get the short shrift when it comes to designing a space that allows for optimum hearing conditions for students and speaking conditions for teachers. Over the last few years, increasing concern from state regulators and facility designers has focused greater attention on improving acoustics. The main acoustical issues to consider…

  19. Parent Hearing Aid Experiences

    ERIC Educational Resources Information Center

    Munoz, Karen; Roberts, Mallory; Mullings, Day; Harward, Richard

    2012-01-01

    This study addresses parent experiences in obtaining and managing hearing aids for their young child. The purpose was to identify challenges parents encounter to determine what state agencies can do to improve parent access to amplification. Data were collected July through September of 2010; 40 parents of children ages birth to 3 years old…

  20. Buying a Hearing Aid

    MedlinePlus

    ... requests or policy questions to our media and public relations staff at newsroom@entnet.org . I don't hear well. What should I do? What should I expect? First, visit a ... use and public sound systems. Other options, such as FM systems ...

  1. Speech and Hearing Therapy.

    ERIC Educational Resources Information Center

    Sakata, Reiko; Sakata, Robert

    1978-01-01

    In the public school, the speech and hearing therapist attempts to foster child growth and development through the provision of services basic to awareness of self and others, management of personal and social interactions, and development of strategies for coping with the handicap. (MM)

  2. Hearing, Listening and Phonosensitivity.

    ERIC Educational Resources Information Center

    Feldman, David

    This paper examines human phonosensitivity (the process by which an organism receives acoustic stimuli and integrates them into its behavior patterns), which is divided into two distinct but inseparable systems: hearing, which controls the reception, transmission, and perception of acoustic stimuli, and listening, which controls the discrimination…

  3. Innovative Technology in Hearing Instruments

    PubMed Central

    2011-01-01

    Hearing instrument technology research is almost entirely focused on the projected needs of the consumer market in the developed world. However, two thirds of the world’s population with hearing impairment live in developing countries and this proportion will increase in future, given present demographic trends. In developing regions, amplification and other hearing health needs may differ from those in industrialized nations, for cultural, health, or economic reasons. World Health Organization estimates indicate that at present only a small percentage of individuals in developing countries who are in need of amplification have access to hearing aid provision. New technologies, such as trainable hearing aids, advanced noise reduction algorithms, feedback reduction circuitry, nano coatings for hearing aid components, and innovative power options, may offer considerable potential benefits, both for individuals with hearing impairment in developing countries and for those who provide hearing health care services in these regions. This article considers the possible supporting role of innovative hearing instrument technologies in the provision of affordable hearing health care services in developing countries and highlights the need for research that considers the requirements of the majority of the world population in need of hearing instrument provision. PMID:22068223

  4. Hearing Loss: Diagnosis and Evaluation.

    PubMed

    Zazove, Philip; Atcherson, Samuel R; Moreland, Christopher; McKee, Michael M

    2015-07-01

    Hearing loss is a common disability in the United States, most frequent among men, elderly individuals, and veterans but is increasingly affecting other younger adults. Types of hearing loss include sensorineural, conductive, and mixed. Hearing loss in children often is related to infections, time spent in a neonatal intensive care unit, and genetic etiologies. Presbycusis (ie, age-related hearing loss) is the most common etiology in adults. Adverse effects of untreated hearing loss include isolation, depression, lower income, and higher unemployment. Hearing aid use reduces levels of disability, cognitive impairment, and psychosocial distress while improving quality of life. At least 75% of individuals with hearing loss are not receiving treatment for it. All infants should be screened for hearing loss, as should children and adults with risk factors. The Joint Commission on Infant Hearing Screening has a 1-3-6 goal for screening: identification by age 1 month, confirmation by age 3 months, and intervention by age 6 months. The presence of an ongoing physician-patient relationship increases the likelihood that a patient will admit to having a hearing loss. Adults can be screened using single-question or standardized instrument screens. All patients with suspected hearing loss should undergo audiometry by an audiology subspecialist. PMID:26161523

  5. The physics of hearing: fluid mechanics and the active process of the inner ear.

    PubMed

    Reichenbach, Tobias; Hudspeth, A J

    2014-07-01

    Most sounds of interest consist of complex, time-dependent admixtures of tones of diverse frequencies and variable amplitudes. To detect and process these signals, the ear employs a highly nonlinear, adaptive, real-time spectral analyzer: the cochlea. Sound excites vibration of the eardrum and the three miniscule bones of the middle ear, the last of which acts as a piston to initiate oscillatory pressure changes within the liquid-filled chambers of the cochlea. The basilar membrane, an elastic band spiraling along the cochlea between two of these chambers, responds to these pressures by conducting a largely independent traveling wave for each frequency component of the input. Because the basilar membrane is graded in mass and stiffness along its length, however, each traveling wave grows in magnitude and decreases in wavelength until it peaks at a specific, frequency-dependent position: low frequencies propagate to the cochlear apex, whereas high frequencies culminate at the base. The oscillations of the basilar membrane deflect hair bundles, the mechanically sensitive organelles of the ear's sensory receptors, the hair cells. As mechanically sensitive ion channels open and close, each hair cell responds with an electrical signal that is chemically transmitted to an afferent nerve fiber and thence into the brain. In addition to transducing mechanical inputs, hair cells amplify them by two means. Channel gating endows a hair bundle with negative stiffness, an instability that interacts with the motor protein myosin-1c to produce a mechanical amplifier and oscillator. Acting through the piezoelectric membrane protein prestin, electrical responses also cause outer hair cells to elongate and shorten, thus pumping energy into the basilar membrane's movements. The two forms of motility constitute an active process that amplifies mechanical inputs, sharpens frequency discrimination, and confers a compressive nonlinearity on responsiveness. These features arise because the

  6. The physics of hearing: fluid mechanics and the active process of the inner ear

    NASA Astrophysics Data System (ADS)

    Reichenbach, Tobias; Hudspeth, A. J.

    2014-07-01

    Most sounds of interest consist of complex, time-dependent admixtures of tones of diverse frequencies and variable amplitudes. To detect and process these signals, the ear employs a highly nonlinear, adaptive, real-time spectral analyzer: the cochlea. Sound excites vibration of the eardrum and the three miniscule bones of the middle ear, the last of which acts as a piston to initiate oscillatory pressure changes within the liquid-filled chambers of the cochlea. The basilar membrane, an elastic band spiraling along the cochlea between two of these chambers, responds to these pressures by conducting a largely independent traveling wave for each frequency component of the input. Because the basilar membrane is graded in mass and stiffness along its length, however, each traveling wave grows in magnitude and decreases in wavelength until it peaks at a specific, frequency-dependent position: low frequencies propagate to the cochlear apex, whereas high frequencies culminate at the base. The oscillations of the basilar membrane deflect hair bundles, the mechanically sensitive organelles of the ear's sensory receptors, the hair cells. As mechanically sensitive ion channels open and close, each hair cell responds with an electrical signal that is chemically transmitted to an afferent nerve fiber and thence into the brain. In addition to transducing mechanical inputs, hair cells amplify them by two means. Channel gating endows a hair bundle with negative stiffness, an instability that interacts with the motor protein myosin-1c to produce a mechanical amplifier and oscillator. Acting through the piezoelectric membrane protein prestin, electrical responses also cause outer hair cells to elongate and shorten, thus pumping energy into the basilar membrane's movements. The two forms of motility constitute an active process that amplifies mechanical inputs, sharpens frequency discrimination, and confers a compressive nonlinearity on responsiveness. These features arise because the

  7. Cognitive Hearing Science

    PubMed Central

    Rudner, Mary; Lunner, Thomas

    2011-01-01

    Stuart Gatehouse was one of the pioneers of cognitive hearing science. The ease of language understanding (ELU) model (Rönnberg) is one example of a cognitive hearing science model where the interplay between memory systems and signal processing is emphasized. The mismatch notion is central to ELU and concerns how phonological information derived from the signal, matches/mismatches phonological representations in lexical and semantic long-term memory (LTM). When signals match, processing is rapid, automatic and implicit, and lexical activation proceeds smoothly. Given a mismatch, lexical activation fails, and working or short-term memory (WM/STM) is assumed to be invoked to engage in explicit repair strategies to disambiguate what was said in the conversation. In a recent study, negative long-term consequences of mismatch were found by means of relating hearing loss to episodic LTM in a sample of old hearing-aid wearers. STM was intact (Rönnberg et al.). Beneficial short-term consequences of a binary masking noise reduction scheme on STM was obtained in 4-talker babble for individuals with high WM capacity, but not in stationary noise backgrounds (Ng et al.). This suggests that individuals high on WM capacity inhibit semantic auditory distraction in 4-talker babble while exploiting the phonological benefits in terms of speech quality provided by binary masking (Wang). Both long-term and short-term mismatch effects, apparent in data sets including behavioral as well as subjective (Rudner et al.) data, need to be taken into account in the design of future hearing instruments. PMID:21606047

  8. Hearing and hearing conservation practices among Australia's professional orchestral musicians.

    PubMed

    O'Brien, Ian; Ackermann, Bronwen J; Driscoll, Tim

    2014-01-01

    Orchestral musicians are an at-risk population for noise-induced hearing loss. Following strategic approaches to mitigate exposure, many must use earplugs to safeguard their hearing, although reported usage rates are poor. Australia has progressive hearing conservation programs within many of its orchestras, yet little is known of earplug usage rates, abilities with earplugs or self-perceived hearing loss in this population. To help direct and inform future approaches to hearing conservation in Australia's orchestras a questionnaire assessing hearing conservation behaviors and the prevalence of self-perceived hearing loss was distributed. A total of 580 musicians across eight professional orchestras were surveyed, with 367 completed surveys (63%) returned. Eighty percent of respondents reported a risk of hearing damage in the orchestra, 64% used earplugs of some type at least some of the time and 83% found this use difficult/impossible. Forty-three percent reported a hearing loss, including 54% in pit orchestras and 46% of those ≤50 years of age. Brass players were least likely to use earplugs, most likely to report usage difficulties and most likely of those ≤50 years of age to report a hearing loss. While earplug usage rates in Australia are encouraging and may be linked to hearing conservation measures in the orchestras, the widespread difficulty reported with the use of these earplugs, the prevalence of self-reported hearing loss and the continued vulnerability of those most at-risk indicate improvements in both earplug design and further education for musicians are required to progress hearing conservation options for this population. PMID:24953885

  9. Bone Anchored Hearing Aid

    PubMed Central

    2002-01-01

    Executive Summary Objective The objective of this health technology policy assessment was to determine the effectiveness and cost-effectiveness of bone-anchored hearing aid (BAHA) in improving the hearing of people with conduction or mixed hearing loss. The Technology The (BAHA) is a bone conduction hearing device that includes a titanium fixture permanently implanted into the mastoid bone of the skull and an external percutaneous sound processor. The sound processor is attached to the fixture by means of a skin penetrating abutment. Because the device bypasses the middle ear and directly stimulates the cochlea, it has been recommended for individuals with conduction hearing loss or discharging middle ear infection. The titanium implant is expected to last a lifetime while the external sound processor is expected to last 5 years. The total initial device cost is approximately $5,300 and the external sound processor costs approximately $3,500. Review of BAHA by the Medical Advisory Secretariat The Medical Advisory Secretariat’s review is a descriptive synthesis of findings from 36 research articles published between January 1990 and May 2002. Summary of Findings No randomized controlled studies were found. The evidence was derived from level 4 case series with relative small sample sizes (ranging from 30-188). The majority of the studies have follow-up periods of eight years or longer. All except one study were based on monaural BAHA implant on the side with the best bone conduction threshold. Safety Level 4 evidence showed that BAHA has been be implanted safely in adults and children with success rates of 90% or higher in most studies. No mortality or life threatening morbidity has been reported. Revision rates for tissue reduction or resiting were generally under 10% for adults but have been reported to be as high as 25% in pediatric studies. Adverse skin reaction around the skin penetration site was the most common complication reported. Most of these

  10. Pediatric Sudden Sensorineural Hearing Loss.

    PubMed

    Kizilay, Ahmet; Koca, Çiğdem Firat

    2016-06-01

    Sudden sensorineural hearing loss is defined as sudden unilateral or bilateral sensorineural hearing loss with at least 30 dB decrease in threshold in 3 contiguous test frequencies occurring over 72 hours or less. It is rare among children. The mechanism of the process and prognosis of the disorder remains unclear. The current incidence of sudden sensorineural hearing loss among pediatric population is unknown. The authors carried out a retrospective chart analysis of patients under 15 years of age from 2004 to 2015, who consulted to the Otolaryngology Head and Neck Surgery Department of Inonu University Medical Faculty. Age, sex, number of affected ear and side, audiometric evaluations, medical follow-up, treatment method, duration of treatment recovery, associated complaints; tinnitus and/or vertigo, presence of mumps disease were recorded for each patient. A 4-frequency pure-tone average (500, 1000, 2000, and 4000 Hz) was calculated for each ear. Complete recovery, defined as some hearing level compared with the nonaffected ear, was observed in 3 patients (21.4 %) and there was no partial hearing recovery. The hearing loss of 11 patient remained unchanged after prednisolone treatment. Two of the 11 patients had bilaterally total sensorineural hearing loss and evaluated as appropriate for cochlear implantation. Sex of patient and laterality of hearing loss were not correlated with hearing recovery. Sensorineural hearing loss among pediatrics has been the issue of otolaryngologists. The incidence, etiology, and treatment methods should be more studied. PMID:27171971

  11. 4 CFR 21.7 - Hearings.

    Code of Federal Regulations, 2010 CFR

    2010-01-01

    ... or on its own initiative, GAO may conduct a hearing in connection with a protest. The request shall set forth the reasons why a hearing is needed to resolve the protest. (b) Prior to the hearing, GAO.... Although hearings ordinarily will be conducted at GAO in Washington, DC, hearings may, at the discretion...

  12. 45 CFR 96.66 - Hearing procedure.

    Code of Federal Regulations, 2013 CFR

    2013-10-01

    ... 45 Public Welfare 1 2013-10-01 2013-10-01 false Hearing procedure. 96.66 Section 96.66 Public Welfare DEPARTMENT OF HEALTH AND HUMAN SERVICES GENERAL ADMINISTRATION BLOCK GRANTS Hearing Procedure § 96.66 Hearing procedure. (a) A hearing is public, except when the Secretary or the presiding officer determines that all or part of a hearing...

  13. 12 CFR 308.160 - Hearings.

    Code of Federal Regulations, 2011 CFR

    2011-01-01

    ... 12 Banks and Banking 4 2011-01-01 2011-01-01 false Hearings. 308.160 Section 308.160 Banks and... Hearings. (a) Hearing dates. The Executive Secretary shall order a hearing to be commenced within 60 days after receipt of a request for hearing on an application filed pursuant to § 308.159. Upon the...

  14. 34 CFR 668.88 - Hearing.

    Code of Federal Regulations, 2011 CFR

    2011-07-01

    ... 34 Education 3 2011-07-01 2011-07-01 false Hearing. 668.88 Section 668.88 Education Regulations of... Proceedings § 668.88 Hearing. (a) A hearing is an orderly presentation of arguments and evidence conducted by a hearing official. (b) If the hearing official, the designated department official who brought...

  15. 36 CFR 1211.620 - Hearings.

    Code of Federal Regulations, 2011 CFR

    2011-07-01

    ... 36 Parks, Forests, and Public Property 3 2011-07-01 2011-07-01 false Hearings. 1211.620 Section... Procedures § 1211.620 Hearings. (a) Opportunity for hearing. Whenever an opportunity for a hearing is... may request of the designated agency official that the matter be scheduled for hearing; or (2)...

  16. 40 CFR 57.807 - Hearing.

    Code of Federal Regulations, 2011 CFR

    2011-07-01

    ... 40 Protection of Environment 5 2011-07-01 2011-07-01 false Hearing. 57.807 Section 57.807... § 57.807 Hearing. (a) Composition of hearing panel. The Presiding Officer shall preside at the hearing held under this subpart. An EPA panel shall also take part in the hearing. In general, the...

  17. 12 CFR 308.160 - Hearings.

    Code of Federal Regulations, 2010 CFR

    2010-01-01

    ... 12 Banks and Banking 4 2010-01-01 2010-01-01 false Hearings. 308.160 Section 308.160 Banks and... Hearings. (a) Hearing dates. The Executive Secretary shall order a hearing to be commenced within 60 days after receipt of a request for hearing on an application filed pursuant to § 308.159. Upon the...

  18. 10 CFR 431.426 - Hearing.

    Code of Federal Regulations, 2011 CFR

    2011-01-01

    ... 10 Energy 3 2011-01-01 2011-01-01 false Hearing. 431.426 Section 431.426 Energy DEPARTMENT OF... § 431.426 Hearing. The Secretary may hold a public hearing, and publish notice in the Federal Register of the date and location of the hearing, when he determines that such a hearing is necessary...

  19. 12 CFR 308.164 - Hearings.

    Code of Federal Regulations, 2011 CFR

    2011-01-01

    ... 12 Banks and Banking 4 2011-01-01 2011-01-01 false Hearings. 308.164 Section 308.164 Banks and... Where a Felony ls Charged § 308.164 Hearings. (a) Hearing dates. The Executive Secretary shall order a hearing to be commenced within 30 days after receipt of a request for hearing filed pursuant to §...

  20. 10 CFR 16.9 - Hearing.

    Code of Federal Regulations, 2010 CFR

    2010-01-01

    ... hearing must conform to procedures contained in the revised FCCS, 31 CFR Chapter IX, 901.3(e). The burden... 10 Energy 1 2010-01-01 2010-01-01 false Hearing. 16.9 Section 16.9 Energy NUCLEAR REGULATORY... § 16.9 Hearing. (a) Request for hearing. (1) An employee shall file a petition for a hearing...

  1. 45 CFR 1203.9 - Hearings.

    Code of Federal Regulations, 2010 CFR

    2010-10-01

    ... 45 Public Welfare 4 2010-10-01 2010-10-01 false Hearings. 1203.9 Section 1203.9 Public Welfare... Hearings. (a) Opportunity for hearing. When an opportunity for a hearing is required by § 1203.8(c... matter be scheduled for hearing; or (2) Advise the applicant or recipient that the matter in question...

  2. 45 CFR 1203.9 - Hearings.

    Code of Federal Regulations, 2011 CFR

    2011-10-01

    ... 45 Public Welfare 4 2011-10-01 2011-10-01 false Hearings. 1203.9 Section 1203.9 Public Welfare... Hearings. (a) Opportunity for hearing. When an opportunity for a hearing is required by § 1203.8(c... matter be scheduled for hearing; or (2) Advise the applicant or recipient that the matter in question...

  3. 18 CFR 1302.9 - Hearings.

    Code of Federal Regulations, 2010 CFR

    2010-04-01

    ... 18 Conservation of Power and Water Resources 2 2010-04-01 2010-04-01 false Hearings. 1302.9... Hearings. (a) Opportunity for hearing. Whenever an opportunity for a hearing is required by § 1302.7(b... notice within which the recipient may request of TVA that the matter be scheduled for hearing or...

  4. 12 CFR 308.155 - Hearing.

    Code of Federal Regulations, 2011 CFR

    2011-01-01

    ... 12 Banks and Banking 4 2011-01-01 2011-01-01 false Hearing. 308.155 Section 308.155 Banks and... Pursuant to Section 32 of the FDIA § 308.155 Hearing. (a) Hearing dates. The Executive Secretary shall order a hearing to be commenced within 30 days after receipt of a request for a hearing filed...

  5. 14 CFR 13.79 - Hearing.

    Code of Federal Regulations, 2010 CFR

    2010-01-01

    ... 14 Aeronautics and Space 1 2010-01-01 2010-01-01 false Hearing. 13.79 Section 13.79 Aeronautics....79 Hearing. If an alleged violator requests a hearing in accordance with § 13.75, the procedure of Subpart D of this part applies. At the close of the hearing, the Hearing Officer, on the record...

  6. 19 CFR 356.23 - Hearing.

    Code of Federal Regulations, 2010 CFR

    2010-04-01

    ... 19 Customs Duties 3 2010-04-01 2010-04-01 false Hearing. 356.23 Section 356.23 Customs Duties... § 356.23 Hearing. (a) Scheduling of hearing. The administrative law judge will schedule the hearing at a... parties adequately to prepare for the hearing and the importance of expeditiously resolving the matter....

  7. 34 CFR 668.88 - Hearing.

    Code of Federal Regulations, 2010 CFR

    2010-07-01

    ... 34 Education 3 2010-07-01 2010-07-01 false Hearing. 668.88 Section 668.88 Education Regulations of... Proceedings § 668.88 Hearing. (a) A hearing is an orderly presentation of arguments and evidence conducted by a hearing official. (b) If the hearing official, the designated department official who brought...

  8. 45 CFR 73b.5 - Hearings.

    Code of Federal Regulations, 2011 CFR

    2011-10-01

    ... 45 Public Welfare 1 2011-10-01 2011-10-01 false Hearings. 73b.5 Section 73b.5 Public Welfare... § 73b.5 Hearings. (a) Hearings shall be stenographically recorded and transcribed and the testimony of witnesses shall be taken under oath or affirmation. Hearings will be closed unless an open hearing...

  9. 39 CFR 957.13 - Hearings.

    Code of Federal Regulations, 2011 CFR

    2011-07-01

    ... 39 Postal Service 1 2011-07-01 2011-07-01 false Hearings. 957.13 Section 957.13 Postal Service... SUSPENSION FROM CONTRACTING § 957.13 Hearings. (a) Hearings are held at 2101 Wilson Boulevard, Suite 600... than 7 days prior to the scheduled date of a hearing, file a request that such hearing be held at...

  10. 45 CFR 16.11 - Hearing.

    Code of Federal Regulations, 2011 CFR

    2011-10-01

    ... 45 Public Welfare 1 2011-10-01 2011-10-01 false Hearing. 16.11 Section 16.11 Public Welfare... BOARD § 16.11 Hearing. (a) Electing a hearing. If the appellant believes a hearing is appropriate, the... appeal file). The Board will approve a request (and may schedule a hearing on its own or in response to...

  11. 32 CFR 195.10 - Hearings.

    Code of Federal Regulations, 2010 CFR

    2010-07-01

    ... 32 National Defense 2 2010-07-01 2010-07-01 false Hearings. 195.10 Section 195.10 National Defense... RIGHTS ACT OF 1964 § 195.10 Hearings. (a) Opportunity for hearing. Whenever an opportunity for a hearing... recipient may request of the responsible Department official that the matter be scheduled for hearing or...

  12. 12 CFR 308.164 - Hearings.

    Code of Federal Regulations, 2010 CFR

    2010-01-01

    ... 12 Banks and Banking 4 2010-01-01 2010-01-01 false Hearings. 308.164 Section 308.164 Banks and... Where a Felony ls Charged § 308.164 Hearings. (a) Hearing dates. The Executive Secretary shall order a hearing to be commenced within 30 days after receipt of a request for hearing filed pursuant to §...

  13. 40 CFR 57.807 - Hearing.

    Code of Federal Regulations, 2010 CFR

    2010-07-01

    ... 40 Protection of Environment 5 2010-07-01 2010-07-01 false Hearing. 57.807 Section 57.807... § 57.807 Hearing. (a) Composition of hearing panel. The Presiding Officer shall preside at the hearing held under this subpart. An EPA panel shall also take part in the hearing. In general, the...

  14. 7 CFR 400.132 - Hearings.

    Code of Federal Regulations, 2011 CFR

    2011-01-01

    ... 7 Agriculture 6 2011-01-01 2011-01-01 false Hearings. 400.132 Section 400.132 Agriculture... Years § 400.132 Hearings. (a) If an employee timely files a petition for a hearing, the FCIC Official will select the date, time, and location for the hearing. (b) The hearing shall be conducted by...

  15. 32 CFR 195.10 - Hearings.

    Code of Federal Regulations, 2011 CFR

    2011-07-01

    ... 32 National Defense 2 2011-07-01 2011-07-01 false Hearings. 195.10 Section 195.10 National Defense... RIGHTS ACT OF 1964 § 195.10 Hearings. (a) Opportunity for hearing. Whenever an opportunity for a hearing... recipient may request of the responsible Department official that the matter be scheduled for hearing or...

  16. 44 CFR 7.13 - Hearings.

    Code of Federal Regulations, 2010 CFR

    2010-10-01

    ... 44 Emergency Management and Assistance 1 2010-10-01 2010-10-01 false Hearings. 7.13 Section 7.13... Programs-General § 7.13 Hearings. (a) Opportunity for hearing. Whenever an opportunity for a hearing is... may request of the responsible agency official that the matter be scheduled for hearing or (2)...

  17. 18 CFR 1302.9 - Hearings.

    Code of Federal Regulations, 2011 CFR

    2011-04-01

    ... 18 Conservation of Power and Water Resources 2 2011-04-01 2011-04-01 false Hearings. 1302.9... Hearings. (a) Opportunity for hearing. Whenever an opportunity for a hearing is required by § 1302.7(b... notice within which the recipient may request of TVA that the matter be scheduled for hearing or...

  18. 45 CFR 73b.5 - Hearings.

    Code of Federal Regulations, 2010 CFR

    2010-10-01

    ... 45 Public Welfare 1 2010-10-01 2010-10-01 false Hearings. 73b.5 Section 73b.5 Public Welfare... § 73b.5 Hearings. (a) Hearings shall be stenographically recorded and transcribed and the testimony of witnesses shall be taken under oath or affirmation. Hearings will be closed unless an open hearing...

  19. 6 CFR 21.15 - Hearings.

    Code of Federal Regulations, 2010 CFR

    2010-01-01

    ... 6 Domestic Security 1 2010-01-01 2010-01-01 false Hearings. 21.15 Section 21.15 Domestic Security... HOMELAND SECURITY § 21.15 Hearings. (a) Opportunity for hearing. Whenever an opportunity for a hearing is... may request of the Secretary that the matter be scheduled for hearing; or (2) Advise the applicant...

  20. 15 CFR 8.12 - Hearings.

    Code of Federal Regulations, 2010 CFR

    2010-01-01

    ... 15 Commerce and Foreign Trade 1 2010-01-01 2010-01-01 false Hearings. 8.12 Section 8.12 Commerce... Compliance § 8.12 Hearings. (a) Opportunity for hearing. Whenever an opportunity for a hearing is required by... hearing, or (2) advise the recipient or other party that the matter in question has been set down...

  1. 37 CFR 11.44 - Hearings.

    Code of Federal Regulations, 2010 CFR

    2010-07-01

    ... 37 Patents, Trademarks, and Copyrights 1 2010-07-01 2010-07-01 false Hearings. 11.44 Section 11.44... Proceedings; Jurisdiction, Sanctions, Investigations, and Proceedings § 11.44 Hearings. (a) The hearing officer shall preside over hearings in disciplinary proceedings. The hearing officer shall set the...

  2. 36 CFR 1211.620 - Hearings.

    Code of Federal Regulations, 2010 CFR

    2010-07-01

    ... 36 Parks, Forests, and Public Property 3 2010-07-01 2010-07-01 false Hearings. 1211.620 Section... Procedures § 1211.620 Hearings. (a) Opportunity for hearing. Whenever an opportunity for a hearing is... may request of the designated agency official that the matter be scheduled for hearing; or (2)...

  3. 10 CFR 205.172 - Hearings.

    Code of Federal Regulations, 2011 CFR

    2011-01-01

    ... 10 Energy 3 2011-01-01 2011-01-01 false Hearings. 205.172 Section 205.172 Energy DEPARTMENT OF ENERGY OIL ADMINISTRATIVE PROCEDURES AND SANCTIONS Conferences, Hearings, and Public Hearings § 205.172 Hearings. (a) The DOE in its discretion may direct that a hearing be convened on its own initiative or...

  4. 49 CFR 209.115 - Hearing.

    Code of Federal Regulations, 2010 CFR

    2010-10-01

    ... 49 Transportation 4 2010-10-01 2010-10-01 false Hearing. 209.115 Section 209.115 Transportation... Hearing. (a) When a hearing is requested and scheduled under § 209.113, a hearing officer designated by the Chief Counsel convenes and presides over the hearing. If requested by respondent and...

  5. 12 CFR 308.155 - Hearing.

    Code of Federal Regulations, 2010 CFR

    2010-01-01

    ... 12 Banks and Banking 4 2010-01-01 2010-01-01 false Hearing. 308.155 Section 308.155 Banks and... Pursuant to Section 32 of the FDIA § 308.155 Hearing. (a) Hearing dates. The Executive Secretary shall order a hearing to be commenced within 30 days after receipt of a request for a hearing filed...

  6. 15 CFR 8.12 - Hearings.

    Code of Federal Regulations, 2011 CFR

    2011-01-01

    ... 15 Commerce and Foreign Trade 1 2011-01-01 2011-01-01 false Hearings. 8.12 Section 8.12 Commerce... Compliance § 8.12 Hearings. (a) Opportunity for hearing. Whenever an opportunity for a hearing is required by... hearing, or (2) advise the recipient or other party that the matter in question has been set down...

  7. 45 CFR 16.11 - Hearing.

    Code of Federal Regulations, 2010 CFR

    2010-10-01

    ... 45 Public Welfare 1 2010-10-01 2010-10-01 false Hearing. 16.11 Section 16.11 Public Welfare... BOARD § 16.11 Hearing. (a) Electing a hearing. If the appellant believes a hearing is appropriate, the... appeal file). The Board will approve a request (and may schedule a hearing on its own or in response to...

  8. 45 CFR 96.66 - Hearing procedure.

    Code of Federal Regulations, 2010 CFR

    2010-10-01

    ... 45 Public Welfare 1 2010-10-01 2010-10-01 false Hearing procedure. 96.66 Section 96.66 Public Welfare DEPARTMENT OF HEALTH AND HUMAN SERVICES GENERAL ADMINISTRATION BLOCK GRANTS Hearing Procedure § 96.66 Hearing procedure. (a) A hearing is public, except when the Secretary or the presiding officer determines that all or part of a hearing...

  9. 5 CFR 1201.52 - Public hearings.

    Code of Federal Regulations, 2013 CFR

    2013-01-01

    ... 5 Administrative Personnel 3 2013-01-01 2013-01-01 false Public hearings. 1201.52 Section 1201.52 Administrative Personnel MERIT SYSTEMS PROTECTION BOARD ORGANIZATION AND PROCEDURES PRACTICES AND PROCEDURES Procedures for Appellate Cases Hearings § 1201.52 Public hearings. (a) Closing the hearing. Hearings are generally open to the...

  10. 10 CFR 205.173 - Public hearings.

    Code of Federal Regulations, 2010 CFR

    2010-01-01

    ... 10 Energy 3 2010-01-01 2010-01-01 false Public hearings. 205.173 Section 205.173 Energy DEPARTMENT OF ENERGY OIL ADMINISTRATIVE PROCEDURES AND SANCTIONS Conferences, Hearings, and Public Hearings § 205.173 Public hearings. (a) A public hearing shall be convened incident to a rulemaking: (1) When the proposed rule or regulation is likely to have...

  11. 18 CFR 808.1 - Public hearings.

    Code of Federal Regulations, 2010 CFR

    2010-04-01

    ... 18 Conservation of Power and Water Resources 2 2010-04-01 2010-04-01 false Public hearings. 808.1... ENFORCEMENT ACTIONS Conduct of Hearings § 808.1 Public hearings. (a) A public hearing shall be conducted in... Commission regulations. (b) A public hearing may be conducted by the Commission in any form or style...

  12. 42 CFR 498.54 - Joint hearings.

    Code of Federal Regulations, 2014 CFR

    2014-10-01

    ... 42 Public Health 5 2014-10-01 2014-10-01 false Joint hearings. 498.54 Section 498.54 Public Health... PROGRAM Hearings § 498.54 Joint hearings. When two or more affected parties have requested hearings and... joint hearings are held, a single record of the proceedings is made and a separate decision issued...

  13. 42 CFR 498.54 - Joint hearings.

    Code of Federal Regulations, 2013 CFR

    2013-10-01

    ... 42 Public Health 5 2013-10-01 2013-10-01 false Joint hearings. 498.54 Section 498.54 Public Health... PROGRAM Hearings § 498.54 Joint hearings. When two or more affected parties have requested hearings and... joint hearings are held, a single record of the proceedings is made and a separate decision issued...

  14. 42 CFR 422.1040 - Joint hearings.

    Code of Federal Regulations, 2012 CFR

    2012-10-01

    ... 42 Public Health 3 2012-10-01 2012-10-01 false Joint hearings. 422.1040 Section 422.1040 Public....1040 Joint hearings. When two or more affected parties have requested hearings and the same or... the prehearing conference or hearing and conduct all proceedings jointly. If joint hearings are...

  15. 42 CFR 423.1040 - Joint hearings.

    Code of Federal Regulations, 2010 CFR

    2010-10-01

    ... 42 Public Health 3 2010-10-01 2010-10-01 false Joint hearings. 423.1040 Section 423.1040 Public... § 423.1040 Joint hearings. When two or more affected parties have requested hearings and the same or... the prehearing conference or hearing and conduct all proceedings jointly. If joint hearings are...

  16. 42 CFR 498.54 - Joint hearings.

    Code of Federal Regulations, 2010 CFR

    2010-10-01

    ... 42 Public Health 5 2010-10-01 2010-10-01 false Joint hearings. 498.54 Section 498.54 Public Health... PROGRAM Hearings § 498.54 Joint hearings. When two or more affected parties have requested hearings and... joint hearings are held, a single record of the preceedings is made and a separate decision issued...

  17. 42 CFR 422.1040 - Joint hearings.

    Code of Federal Regulations, 2014 CFR

    2014-10-01

    ... 42 Public Health 3 2014-10-01 2014-10-01 false Joint hearings. 422.1040 Section 422.1040 Public....1040 Joint hearings. When two or more affected parties have requested hearings and the same or... the prehearing conference or hearing and conduct all proceedings jointly. If joint hearings are...

  18. 42 CFR 498.54 - Joint hearings.

    Code of Federal Regulations, 2012 CFR

    2012-10-01

    ... 42 Public Health 5 2012-10-01 2012-10-01 false Joint hearings. 498.54 Section 498.54 Public Health... PROGRAM Hearings § 498.54 Joint hearings. When two or more affected parties have requested hearings and... joint hearings are held, a single record of the preceedings is made and a separate decision issued...

  19. 42 CFR 423.1040 - Joint hearings.

    Code of Federal Regulations, 2011 CFR

    2011-10-01

    ... 42 Public Health 3 2011-10-01 2011-10-01 false Joint hearings. 423.1040 Section 423.1040 Public... § 423.1040 Joint hearings. When two or more affected parties have requested hearings and the same or... the prehearing conference or hearing and conduct all proceedings jointly. If joint hearings are...

  20. 42 CFR 498.54 - Joint hearings.

    Code of Federal Regulations, 2011 CFR

    2011-10-01

    ... 42 Public Health 5 2011-10-01 2011-10-01 false Joint hearings. 498.54 Section 498.54 Public Health... PROGRAM Hearings § 498.54 Joint hearings. When two or more affected parties have requested hearings and... joint hearings are held, a single record of the preceedings is made and a separate decision issued...

  1. 42 CFR 422.1040 - Joint hearings.

    Code of Federal Regulations, 2013 CFR

    2013-10-01

    ... 42 Public Health 3 2013-10-01 2013-10-01 false Joint hearings. 422.1040 Section 422.1040 Public....1040 Joint hearings. When two or more affected parties have requested hearings and the same or... the prehearing conference or hearing and conduct all proceedings jointly. If joint hearings are...

  2. 10 CFR 205.173 - Public hearings.

    Code of Federal Regulations, 2012 CFR

    2012-01-01

    ... 10 Energy 3 2012-01-01 2012-01-01 false Public hearings. 205.173 Section 205.173 Energy DEPARTMENT OF ENERGY OIL ADMINISTRATIVE PROCEDURES AND SANCTIONS Conferences, Hearings, and Public Hearings § 205.173 Public hearings. (a) A public hearing shall be convened incident to a rulemaking: (1) When...

  3. 12 CFR 1081.300 - Public hearings.

    Code of Federal Regulations, 2013 CFR

    2013-01-01

    ... 12 Banks and Banking 8 2013-01-01 2013-01-01 false Public hearings. 1081.300 Section 1081.300... Hearings § 1081.300 Public hearings. All hearings in adjudication proceedings shall be public unless a... Director on the grounds that holding an open hearing would be contrary to the public interest....

  4. 12 CFR 1081.300 - Public hearings.

    Code of Federal Regulations, 2012 CFR

    2012-01-01

    ... 12 Banks and Banking 8 2012-01-01 2012-01-01 false Public hearings. 1081.300 Section 1081.300... Hearings § 1081.300 Public hearings. All hearings in adjudication proceedings shall be public unless a... Director on the grounds that holding an open hearing would be contrary to the public interest....

  5. 10 CFR 205.173 - Public hearings.

    Code of Federal Regulations, 2014 CFR

    2014-01-01

    ... 10 Energy 3 2014-01-01 2014-01-01 false Public hearings. 205.173 Section 205.173 Energy DEPARTMENT OF ENERGY OIL ADMINISTRATIVE PROCEDURES AND SANCTIONS Conferences, Hearings, and Public Hearings § 205.173 Public hearings. (a) A public hearing shall be convened incident to a rulemaking: (1) When...

  6. 18 CFR 808.1 - Public hearings.

    Code of Federal Regulations, 2011 CFR

    2011-04-01

    ... 18 Conservation of Power and Water Resources 2 2011-04-01 2011-04-01 false Public hearings. 808.1... ENFORCEMENT ACTIONS Conduct of Hearings § 808.1 Public hearings. (a) A public hearing shall be conducted in... Commission regulations. (b) A public hearing may be conducted by the Commission in any form or style...

  7. 49 CFR 209.115 - Hearing.

    Code of Federal Regulations, 2011 CFR

    2011-10-01

    ... 49 Transportation 4 2011-10-01 2011-10-01 false Hearing. 209.115 Section 209.115 Transportation... Hearing. (a) When a hearing is requested and scheduled under § 209.113, a hearing officer designated by the Chief Counsel convenes and presides over the hearing. If requested by respondent and...

  8. 29 CFR 1450.22 - Hearing.

    Code of Federal Regulations, 2010 CFR

    2010-07-01

    ... 29 Labor 4 2010-07-01 2010-07-01 false Hearing. 1450.22 Section 1450.22 Labor Regulations Relating... STATES Salary Offset § 1450.22 Hearing. (a) Petition for hearing. (1) A hearing may be requested by..., and is not accepted pursuant to paragraph (a)(4) of this section, the employee's right to hearing...

  9. 28 CFR 42.109 - Hearings.

    Code of Federal Regulations, 2010 CFR

    2010-07-01

    ... 28 Judicial Administration 1 2010-07-01 2010-07-01 false Hearings. 42.109 Section 42.109 Judicial....109 Hearings. (a) Opportunity for hearing. Whenever an opportunity for a hearing is required by § 42... that the responsible Department official schedule the matter for hearing, or (2) advise the...

  10. 22 CFR 34.15 - Outside hearings.

    Code of Federal Regulations, 2011 CFR

    2011-04-01

    ... the origin, nature, and amount of the debt; (ii) The hearing official's findings, analysis, and... hearings shall be conducted by a hearing official not under the supervision or control of STATE. (c) Procedure. (1) After the employee requests a hearing, the hearing official shall notify the employee of...

  11. Idiopathic Sudden Sensorineural Hearing Loss With Minimal Hearing Impairment

    PubMed Central

    Cho, Chin Saeng

    2015-01-01

    Objectives The aim of the study was to determine the characteristics of patients who did not match the audiometric criteria of idiopathic sudden sensorineural hearing loss (SSNHL) but complained of acute hearing loss. Methods By thorough medical chart reviews, historical cohort study was performed with consecutive data of 589 patients complaining of acute unilateral sensorineural hearing loss without identifiable causes between 2005 and 2013. Those patients demonstrating a hearing loss of at least 30 dB at three consecutive frequencies based on pure tone audiometry were classified as group I; the others were classified as group II. Patients' characteristics, final hearing, and hearing improvement rate (HIR) between the two groups were compared. Results Group II exhibited distinctive characteristics, including an early age of onset of the hearing loss (P<0.01), an absence of accompanying diabetes (P<0.01) and hypertension (P<0.01), and better unaffected hearing and final hearing compared with group I (P<0.001). However, the HIR of the patients in the two groups was not significantly different (P>0.05). Conclusion Patients who did not meet the audiological criteria of SSNHL exhibited distinctive characteristics compared to SSNHL patients. PMID:26622953

  12. Magnesium and hearing.

    PubMed

    Cevette, Michael J; Vormann, Jürgen; Franz, Kay

    2003-01-01

    The last several decades have revealed clinical and experimental data regarding the importance of magnesium (Mg) in hearing. Increased susceptibility to noise damage, ototoxicity, and auditory hyperexcitability are linked to states of Mg deficiency. Evidence for these processes has come slowly and direct effects have remained elusive because plasma Mg levels do not always correlate with its deficiency. Despite the major progress in the understanding of cochlear mechanical and auditory nerve function, the neurochemical and pharmacologic role of Mg is not clear. The putative mechanism suggests that Mg deficiency may contribute to a metabolic cellular cascade of events. Mg deficiency leads to an increased permeability of the calcium channel in the hair cells with a consequent over influx of calcium, an increased release of glutamate via exocytosis, and over stimulation of NMDA receptors on the auditory nerve. This paper provides a current overview of relevant Mg metabolism and deficiency and its influence on hearing. PMID:12940704

  13. Directional Hearing Aid

    NASA Technical Reports Server (NTRS)

    Jhabvala, M.; Lin, H. C.

    1989-01-01

    Hearing-aid device indicates visually whether sound is coming from left, right, back, or front. Device intended to assist individuals who are deaf in at least one ear and unable to discern naturally directions to sources of sound. Device promotes safety in street traffic, on loading docks, and in presence of sirens, alarms, and other warning sounds. Quadraphonic version of device built into pair of eyeglasses and binaural version built into visor.

  14. Frequency-Shift Hearing Aid

    NASA Technical Reports Server (NTRS)

    Weinstein, Leonard M.

    1994-01-01

    Proposed hearing aid maps spectrum of speech into band of lower frequencies at which ear remains sensitive. By redirecting normal speech frequencies into frequency band from 100 to 1,500 Hz, hearing aid allows people to understand normal conversation, including telephone calls. Principle operation of hearing aid adapted to other uses such as, clearing up noisy telephone or radio communication. In addition, loud-speakers more easily understood in presence of high background noise.

  15. Hearing Loss in Children: Screening and Diagnosis

    MedlinePlus

    ... Form Controls NCBDDD Cancel Submit Search The CDC Hearing Loss in Children Note: Javascript is disabled or is ... message, please visit this page: About CDC.gov . Hearing Loss Homepage Facts Noise-Induced Hearing Loss Genetics of ...

  16. 19 CFR 111.67 - Hearing.

    Code of Federal Regulations, 2010 CFR

    2010-04-01

    ... Cancellation, Suspension, or Revocation of License or Permit, and Monetary Penalty in Lieu of Suspension or Revocation § 111.67 Hearing. (a) Hearing officer. The hearing officer must be an administrative law...

  17. 10 CFR 501.34 - Public hearing.

    Code of Federal Regulations, 2011 CFR

    2011-01-01

    ... transcript made of the public hearing. The hearing record shall remain open for a period of fourteen (14... its discretion, re-open the hearing at the request of a party or participant, to permit...

  18. Consanguinity and hereditary hearing loss in Qatar.

    PubMed

    Girotto, Giorgia; Mezzavilla, Massimo; Abdulhadi, Khalid; Vuckovic, Dragana; Vozzi, Diego; Khalifa Alkowari, Moza; Gasparini, Paolo; Badii, Ramin

    2014-01-01

    Qatar is a sovereign state located on the Eastern coast of the Arabian Peninsula in the Persian Gulf. Its native population consists of 3 major subgroups: people of Arabian origin or Bedouins, those from an Eastern or Persian ancestry and individuals with African admixture. Historically, all types of consanguineous marriages have been and still are common in the Qatari population, particularly among first and double-first cousins. Thus, there is a higher risk for most inherited diseases including hereditary hearing loss (HHL). In particular, a hearing loss prevalence of 5.2% has been reported in Qatar, with parental consanguinity being more common among affected individuals as compared with unaffected ones. Our recent molecular results confirm a high homogeneity and level of inbreeding in Qatari HHL patients. Among all HHL genes, GJB2, the major player worldwide, accounts for a minor proportion of cases and at least 3 additional genes have been found to be mutated in Qatari patients. Interestingly, one gene, BDP1, has been described to cause HHL only in this country. These results point towards an unexpected level of genetic heterogeneity despite the high level of inbreeding. This review provides an up-to-date picture of HHL in Qatar and of the impact of consanguinity on this disease. PMID:25060281

  19. Middle Ear Infection (Chronic Otitis Media) and Hearing Loss

    MedlinePlus

    ... You Middle Ear Infection (Chronic Otitis Media) and Hearing Loss Middle Ear Infection (Chronic Otitis Media) and Hearing ... learning important speech and language skills. Types of hearing loss Conductive hearing loss is a form of hearing ...

  20. Deaf and Hearing Parents' Interactions with Eldest Hearing Children.

    ERIC Educational Resources Information Center

    Jones, Elaine G.; Dumas, Robert E.

    1996-01-01

    This study compared interactions in 18 families headed by either deaf or hearing parents with an eldest hearing child (aged 7 to 11). Transcripts of parent/child dyadic interactions during a vacation planning activity were analyzed. There were no significant differences between children in the two groups, though deaf parents demonstrated less…

  1. 20 CFR 410.630 - Hearing; right to hearing.

    Code of Federal Regulations, 2010 CFR

    2010-04-01

    ... 20 Employees' Benefits 2 2010-04-01 2010-04-01 false Hearing; right to hearing. 410.630 Section 410.630 Employees' Benefits SOCIAL SECURITY ADMINISTRATION FEDERAL COAL MINE HEALTH AND SAFETY ACT OF 1969, TITLE IV-BLACK LUNG BENEFITS (1969- ) Determinations of Disability, Other...

  2. Hearing symptoms personal stereos

    PubMed Central

    da Luz, Tiara Santos; Borja, Ana Lúcia Vieira de Freitas

    2012-01-01

    Summary Introduction: Practical and portable the personal stereos if had become almost indispensable accessories in the day the day. Studies disclose that the portable players of music can cause auditory damages in the long run for who hear music in high volume for a drawn out time. Objective: to verify the prevalence of auditory symptoms in users of amplified players and to know its habits of use Method: Observational prospective study of transversal cut carried through in three institutions of education of the city of Salvador BA, being two of public net and one of the private net. 400 students had answered to the questionnaire, of both the sex, between 14 and 30 years that had related the habit to use personal stereos. Results: The symptoms most prevalent had been hyperacusis (43.5%), auricular fullness (30.5%) and humming (27.5), being that the humming is the symptom most present in the population youngest. How much to the daily habits: 62.3% frequent use, 57% in raised intensities, 34% in drawn out periods. An inverse relation between exposition time was verified and the band of age (p = 0,000) and direct with the prevalence of the humming. Conclusion: Although to admit to have knowledge on the damages that the exposition the sound of high intensity can cause the hearing, the daily habits of the young evidence the inadequate use of the portable stereos characterized by long periods of exposition, raised intensities, frequent use and preference for the insertion phones. The high prevalence of symptoms after the use suggests a bigger risk for the hearing of these young. PMID:25991931

  3. Noise-induced hearing loss.

    PubMed

    Catlin, F I

    1986-03-01

    Hearing loss affects 30 million people in the United States; of these, 21 million are over the age of 65 years. This disorder may have several causes: heredity, noise, aging, and disease. Hearing loss from noise has been recognized for centuries but was generally ignored until some time after the Industrial Revolution. Hearing loss from occupational exposure to hazardous noise was identified as a compensable disability by the United States courts in 1948 to 1959. Development of noisy jet engines and supersonic aircraft created additional claims for personal and property damage in the 1950s and 1960s. These conditions led to legislation for noise control in the form of the Occupational Safety and Health Act of 1970 and the Noise Control Act of 1972. Protection of the noise-exposed employee was also an objective of the Hearing Conservation Act of 1971. Subsequent studies have confirmed the benefits of periodic hearing tests for workers exposed to hazardous noise and of otologic evaluation as part of the hearing conservation process. Research studies in laboratory animals, using scanning electron microscopical techniques, have demonstrated that damage to the inner ear and organ of hearing can occur even though subjective (conditioned) response to sound stimuli remains unaffected. Some investigators have employed an epidemiologic approach to identify risk factors and to develop profiles to susceptibility to noise-induced hearing loss. The need for joint involvement of workers and employers in the reduction and control of occupational noise hazards is evident. PMID:2938482

  4. Intellectual Disabilities and Hearing Loss

    ERIC Educational Resources Information Center

    Herer, Gilbert R.

    2012-01-01

    Undetected/untreated hearing loss imposes significant limitations upon individuals with intellectual disabilities (ID). It can interfere with cognitive development, impede communicative and social interactions, and limit vocational aspirations. Over the past decade, the hearing of 9961 people with ID was evaluated at Special Olympics sports…

  5. The Stigma of Hearing Loss

    ERIC Educational Resources Information Center

    Wallhagen, Margaret I.

    2010-01-01

    Purpose: To explore dimensions of stigma experienced by older adults with hearing loss and those with whom they frequently communicate to target interventions promoting engagement and positive aging. Design and Methods: This longitudinal qualitative study conducted interviews over 1 year with dyads where one partner had hearing loss. Participants…

  6. A Hearing Aid Primer 1

    ERIC Educational Resources Information Center

    Yetter, Carol J.

    2009-01-01

    This hearing aid primer is designed to define the differences among the three levels of hearing instrument technology: conventional analog circuit technology (most basic), digitally programmable/analog circuit technology (moderately advanced), and fully digital technology (most advanced). Both moderate and advanced technologies mean that hearing…

  7. Noise-induced hearing loss

    SciTech Connect

    Catlin, F.I.

    1986-03-01

    Hearing loss affects 30 million people in the United States; of these, 21 million are over the age of 65 years. This disorder may have several causes: heredity, noise, aging, and disease. Hearing loss from noise has been recognized for centuries but was generally ignored until some time after the Industrial Revolution. Hearing loss from occupational exposure to hazardous noise was identified as a compensable disability by the United States courts in 1948 to 1959. Development of noisy jet engines and supersonic aircraft created additional claims for personal and property damage in the 1950s and 1960s. These conditions led to legislation for noise control in the form of the Occupational Safety and Health Act of 1970 and the Noise Control Act of 1972. Protection of the noise-exposed employee was also an objective of the Hearing Conservation Act of 1971. Subsequent studies have confirmed the benefits of periodic hearing tests for workers exposed to hazardous noise and of otologic evaluation as part of the hearing conservation process. Research studies in laboratory animals, using scanning electron microscopical techniques, have demonstrated that damage to the inner ear and organ of hearing can occur even though subjective (conditioned) response to sound stimuli remains unaffected. Some investigators have employed an epidemiologic approach to identify risk factors and to develop profiles to susceptibility to noise-induced hearing loss. The need for joint involvement of workers and employers in the reduction and control of occupational noise hazards is evident. 19 references.

  8. 77 FR 23319 - Public Hearing

    Federal Register 2010, 2011, 2012, 2013, 2014

    2012-04-18

    ... From the Federal Register Online via the Government Publishing Office SUSQUEHANNA RIVER BASIN COMMISSION Public Hearing AGENCY: Susquehanna River Basin Commission. ACTION: Notice. SUMMARY: The Susquehanna River Basin Commission will hold a public hearing on May 10, 2012, in Harrisburg, Pennsylvania....

  9. Articulation index and hearing handicap.

    PubMed

    Holcomb, L M; Nerbonne, M A; Konkle, D F

    2000-04-01

    This investigation examined the relationship between perceived hearing handicap and the Articulation Index (AI) and the extent to which this relationship was influenced by the variables age, gender, degree of hearing loss, and audiometric slope. Subject age, gender, pure-tone thresholds, and scores for the Self-Assessment of Communication (SAC) and the Significant Other Assessment of Communication (SOAC) were extracted retrospectively from 373 patient files (194 males, 179 females). Correlation analysis revealed a significant (p < .01) negative relationship between AI values and both measures of hearing handicap, and also indicated that SAC/SOAC total scores correlated significantly (p < .01) with each other. Partial correlation analyses revealed that degree of hearing loss was the only variable under study that had substantial influence on the strength of AI/hearing handicap correlations. PMID:10783925

  10. Hearing aid malfunction detection system

    NASA Technical Reports Server (NTRS)

    Kessinger, R. L. (Inventor)

    1977-01-01

    A malfunction detection system for detecting malfunctions in electrical signal processing circuits is disclosed. Malfunctions of a hearing aid in the form of frequency distortion and/or inadequate amplification by the hearing aid amplifier, as well as weakening of the hearing aid power supply are detectable. A test signal is generated and a timed switching circuit periodically applies the test signal to the input of the hearing aid amplifier in place of the input signal from the microphone. The resulting amplifier output is compared with the input test signal used as a reference signal. The hearing aid battery voltage is also periodically compared to a reference voltage. Deviations from the references beyond preset limits cause a warning system to operate.

  11. A rare novel mutation in TECTA causes autosomal dominant nonsyndromic hearing loss in a Mongolian family

    PubMed Central

    2014-01-01

    Background The genetic basis of autosomal dominant nonsyndromic hearing loss is complex. Genetic factors are responsible for approximately 50% of cases with congenital hearing loss. However, no previous studies have documented the clinical phenotype and genetic basis of autosomal dominant nonsyndromic hearing loss in Mongolians. Methods In this study, we performed exon capture sequencing of a Mongolian family with hereditary hearing loss and identified a novel mutation in TECTA gene, which encodes α -tectorin, a major component of the inner ear extracellular matrix that contacts the specialized sensory hair cells. Results The novel G → T missense mutation at nucleotide 6016 results in a substitution of amino acid aspartate at 2006 with tyrosine (Asp2006Tyr) in a highly conserved zona pellucida (ZP) domain of α-tectorin. The mutation is not found in control subjects from the same family with normal hearing and a genotype-phenotype correlation is observed. Conclusion A novel missense mutation c.6016 G > T (p.Asp2006Tyr) of TECTA gene is a characteristic TECTA-related mutation which causes autosomal dominant nonsyndromic hearing loss. Our result indicated that mutation in TECTA gene is responsible for the hearing loss in this Mongolian family. PMID:25008054

  12. [Advances in hereditary hearing loss caused by TMC1 mutations].

    PubMed

    Wu, Kaiwen; Wang, Hongyang; Wang, Qiuju

    2016-03-01

    Hearing loss is the most frequent sensorineural disorder worldwild, among which about 50% are caused by genetic factors. TMC1 is one of the common genes causing hereditary hearing loss. TMC1 mutations can cause pre-lingual profound/severe autosomal recessive (DFNB7/11) and post-lingual progressive autosomal dominant (DFNA36) non-syndromic hearing loss. Murine models studies show that TMC1, 2 are expressed in cochlea inner and outer hair cells and maintain normal mechanoelectrical transduction (MET) functions of the hair cells. A growing number of evidence indicate that TMC1, 2 are components of the MET complex. It is necessary to definite the precise distribution and exact function of TMC1, 2, because it is important to understand the regulating mechanism of auditory function. PMID:27033582

  13. 46 CFR 502.144 - Notice of time and place of hearing; postponement of hearing.

    Code of Federal Regulations, 2010 CFR

    2010-10-01

    ... 46 Shipping 9 2010-10-01 2010-10-01 false Notice of time and place of hearing; postponement of... and place of hearing; postponement of hearing. (a) Notice of hearing will designate the time and place..., or electronic mail. (b) Motions for postponement of any hearing date shall be filed in...

  14. 40 CFR 24.14 - Scheduling the hearing; pre-hearing submissions by the parties.

    Code of Federal Regulations, 2010 CFR

    2010-07-01

    ... 40 Protection of Environment 1 2010-07-01 2010-07-01 false Scheduling the hearing; pre-hearing submissions by the parties. 24.14 Section 24.14 Protection of Environment ENVIRONMENTAL PROTECTION AGENCY... Hearings on Orders Requiring Corrective Measures § 24.14 Scheduling the hearing; pre-hearing submissions...

  15. 20 CFR 416.1415 - Disability hearing-disability hearing officers.

    Code of Federal Regulations, 2014 CFR

    2014-04-01

    ... 20 Employees' Benefits 2 2014-04-01 2014-04-01 false Disability hearing-disability hearing... Reopening of Determinations and Decisions Reconsideration § 416.1415 Disability hearing—disability hearing officers. (a) General. Your disability hearing will be conducted by a disability hearing officer who...

  16. 20 CFR 416.1415 - Disability hearing-disability hearing officers.

    Code of Federal Regulations, 2011 CFR

    2011-04-01

    ... 20 Employees' Benefits 2 2011-04-01 2011-04-01 false Disability hearing-disability hearing... Reopening of Determinations and Decisions Reconsideration § 416.1415 Disability hearing—disability hearing officers. (a) General. Your disability hearing will be conducted by a disability hearing officer who...

  17. 20 CFR 416.1415 - Disability hearing-disability hearing officers.

    Code of Federal Regulations, 2012 CFR

    2012-04-01

    ... 20 Employees' Benefits 2 2012-04-01 2012-04-01 false Disability hearing-disability hearing... Reopening of Determinations and Decisions Reconsideration § 416.1415 Disability hearing—disability hearing officers. (a) General. Your disability hearing will be conducted by a disability hearing officer who...

  18. 20 CFR 416.1415 - Disability hearing-disability hearing officers.

    Code of Federal Regulations, 2013 CFR

    2013-04-01

    ... 20 Employees' Benefits 2 2013-04-01 2013-04-01 false Disability hearing-disability hearing... Reopening of Determinations and Decisions Reconsideration § 416.1415 Disability hearing—disability hearing officers. (a) General. Your disability hearing will be conducted by a disability hearing officer who...

  19. 20 CFR 416.1415 - Disability hearing-disability hearing officers.

    Code of Federal Regulations, 2010 CFR

    2010-04-01

    ... 20 Employees' Benefits 2 2010-04-01 2010-04-01 false Disability hearing-disability hearing... Reopening of Determinations and Decisions Reconsideration § 416.1415 Disability hearing—disability hearing officers. (a) General. Your disability hearing will be conducted by a disability hearing officer who...

  20. Hearing aid fitting in older persons with hearing impairment: the influence of cognitive function, age, and hearing loss on hearing aid benefit

    PubMed Central

    Meister, Hartmut; Rählmann, Sebastian; Walger, Martin; Margolf-Hackl, Sabine; Kießling, Jürgen

    2015-01-01

    Purpose To examine the association of cognitive function, age, and hearing loss with clinically assessed hearing aid benefit in older hearing-impaired persons. Methods Hearing aid benefit was assessed using objective measures regarding speech recognition in quiet and noisy environments as well as a subjective measure reflecting everyday situations captured using a standardized questionnaire. A broad range of general cognitive functions such as attention, memory, and intelligence were determined using different neuropsychological tests. Linear regression analyses were conducted with the outcome of the neuropsychological tests as well as age and hearing loss as independent variables and the benefit measures as dependent variables. Thirty experienced older hearing aid users with typical age-related hearing impairment participated. Results Most of the benefit measures revealed that the participants obtained significant improvement with their hearing aids. Regression models showed a significant relationship between a fluid intelligence measure and objective hearing aid benefit. When individual hearing thresholds were considered as an additional independent variable, hearing loss was the only significant contributor to the benefit models. Lower cognitive capacity – as determined by the fluid intelligence measure – was significantly associated with greater hearing loss. Subjective benefit could not be predicted by any of the variables considered. Conclusion The present study does not give evidence that hearing aid benefit is critically associated with cognitive function in experienced hearing aid users. However, it was found that lower fluid intelligence scores were related to higher hearing thresholds. Since greater hearing loss was associated with a greater objective benefit, these results strongly support the advice of using hearing aids regardless of age and cognitive function to counter hearing loss and the adverse effects of age-related hearing impairment. Still

  1. Help! Libraries and the Hard of Hearing.

    ERIC Educational Resources Information Center

    1982

    This transcript contains the text of three speeches on the problems of people with hearing impairments and the provision of library services to the hard of hearing. Howard Edward (Rocky) Stone, founder of Self-Help-For-Hearing-Impaired-People, Inc., describes his difficulties as a hearing impaired person, the growing number of Americans with…

  2. 5 CFR 1215.5 - Hearing.

    Code of Federal Regulations, 2013 CFR

    2013-01-01

    ... Federal Claims Collection Standards 4 CFR 102.3(c). The burden shall be on the employee to demonstrate... 5 Administrative Personnel 3 2013-01-01 2013-01-01 false Hearing. 1215.5 Section 1215.5... § 1215.5 Hearing. (a) Request for hearing. (1) An employee must file a petition for a hearing...

  3. 5 CFR 1215.5 - Hearing.

    Code of Federal Regulations, 2012 CFR

    2012-01-01

    ... Federal Claims Collection Standards 4 CFR 102.3(c). The burden shall be on the employee to demonstrate... 5 Administrative Personnel 3 2012-01-01 2012-01-01 false Hearing. 1215.5 Section 1215.5... § 1215.5 Hearing. (a) Request for hearing. (1) An employee must file a petition for a hearing...

  4. 10 CFR 110.100 - Public hearings.

    Code of Federal Regulations, 2010 CFR

    2010-01-01

    ... 10 Energy 2 2010-01-01 2010-01-01 false Public hearings. 110.100 Section 110.100 Energy NUCLEAR REGULATORY COMMISSION (CONTINUED) EXPORT AND IMPORT OF NUCLEAR EQUIPMENT AND MATERIAL Hearings § 110.100 Public hearings. Hearings under this part will be public unless the Commission directs otherwise....

  5. 10 CFR 110.102 - Hearing docket.

    Code of Federal Regulations, 2010 CFR

    2010-01-01

    ... 10 Energy 2 2010-01-01 2010-01-01 false Hearing docket. 110.102 Section 110.102 Energy NUCLEAR REGULATORY COMMISSION (CONTINUED) EXPORT AND IMPORT OF NUCLEAR EQUIPMENT AND MATERIAL Hearings § 110.102 Hearing docket. For each hearing, the Secretary will maintain a docket which will include the...

  6. 12 CFR 509.301 - Hearing procedures.

    Code of Federal Regulations, 2011 CFR

    2011-01-01

    ... procedures. (a) Hearings. The following procedures apply to hearings under 12 CFR part 585. (1) The hearing... counsel and the applicant. (4) 12 CFR 509.2, 509.4, 509.6 through 509.12, and 509.16 apply to the hearing... the proceeding is being conducted. Witness fees are paid in accordance with 12 CFR 509.14. (9)...

  7. 12 CFR 509.301 - Hearing procedures.

    Code of Federal Regulations, 2013 CFR

    2013-01-01

    ... procedures. (a) Hearings. The following procedures apply to hearings under 12 CFR part 585. (1) The hearing... counsel and the applicant. (4) 12 CFR 509.2, 509.4, 509.6 through 509.12, and 509.16 apply to the hearing... the proceeding is being conducted. Witness fees are paid in accordance with 12 CFR 509.14. (9)...

  8. 42 CFR 93.211 - Hearing.

    Code of Federal Regulations, 2011 CFR

    2011-10-01

    ... 42 Public Health 1 2011-10-01 2011-10-01 false Hearing. 93.211 Section 93.211 Public Health PUBLIC... Definitions § 93.211 Hearing. Hearing means that part of the research misconduct proceeding from the time a respondent files a request for an administrative hearing to contest ORI findings of research misconduct...

  9. 32 CFR 723.5 - Hearing.

    Code of Federal Regulations, 2011 CFR

    2011-07-01

    ... 32 National Defense 5 2011-07-01 2011-07-01 false Hearing. 723.5 Section 723.5 National Defense... § 723.5 Hearing. (a) Convening of board. The Board will convene, recess and adjourn at the call of the Chair or Acting Chair. (b) Conduct of hearing. (1) The hearing shall be conducted by the Chair or...

  10. 20 CFR 901.44 - Hearings.

    Code of Federal Regulations, 2010 CFR

    2010-04-01

    ... 20 Employees' Benefits 3 2010-04-01 2010-04-01 false Hearings. 901.44 Section 901.44 Employees... § 901.44 Hearings. (a) In general. The Administrative Law Judge shall preside at the hearing on a complaint for the suspension or termination of an enrolled actuary. Hearings shall be...

  11. 9 CFR 202.5 - Hearing.

    Code of Federal Regulations, 2011 CFR

    2011-01-01

    ... 9 Animals and Animal Products 2 2011-01-01 2011-01-01 false Hearing. 202.5 Section 202.5 Animals... STOCKYARDS ACT Rules of Practice Applicable to Rate Proceedings § 202.5 Hearing. The hearing will be oral unless all parties waive oral hearing. It will be written if not oral. Notice of the date, time and...

  12. 43 CFR 17.8 - Hearings.

    Code of Federal Regulations, 2010 CFR

    2010-10-01

    ... 43 Public Lands: Interior 1 2010-10-01 2010-10-01 false Hearings. 17.8 Section 17.8 Public Lands... DEPARTMENT OF THE INTERIOR Nondiscrimination on the Basis of Race, Color, or National Origin § 17.8 Hearings. (a) Opportunity for hearing. Whenever an opportunity for a hearing is required by §...

  13. 34 CFR 668.116 - Hearing.

    Code of Federal Regulations, 2010 CFR

    2010-07-01

    ... 34 Education 3 2010-07-01 2010-07-01 false Hearing. 668.116 Section 668.116 Education Regulations... Program Review Determinations § 668.116 Hearing. (a) A hearing is a process conducted by the hearing official whereby an orderly presentation of arguments and evidence is made by the parties. (b) The...

  14. 22 CFR 209.9 - Hearings.

    Code of Federal Regulations, 2010 CFR

    2010-04-01

    ... 22 Foreign Relations 1 2010-04-01 2010-04-01 false Hearings. 209.9 Section 209.9 Foreign Relations... INTERNATIONAL DEVELOPMENT-EFFECTUATION OF TITLE VI OF THE CIVIL RIGHTS ACT OF 1964 § 209.9 Hearings. (a) Opportunity for hearing. Whenever an opportunity for a hearing is required by § 209.8(c), reasonable...

  15. 29 CFR 101.34 - Hearing.

    Code of Federal Regulations, 2010 CFR

    2010-07-01

    ... 29 Labor 2 2010-07-01 2010-07-01 false Hearing. 101.34 Section 101.34 Labor Regulations Relating... Section 10(k) of the Act § 101.34 Hearing. If the parties have not adjusted the dispute or agreed upon methods of voluntary adjustment, a hearing, usually open to the public, is held before a hearing...

  16. 45 CFR 33.6 - Hearings.

    Code of Federal Regulations, 2011 CFR

    2011-10-01

    ... 45 Public Welfare 1 2011-10-01 2011-10-01 false Hearings. 33.6 Section 33.6 Public Welfare DEPARTMENT OF HEALTH AND HUMAN SERVICES GENERAL ADMINISTRATION SALARY OFFSET § 33.6 Hearings. (a) Petitions for hearing. (1) To request a hearing concerning the existence or amount of the debt or the...

  17. 29 CFR 6.54 - Hearing.

    Code of Federal Regulations, 2011 CFR

    2011-07-01

    ... 29 Labor 1 2011-07-01 2011-07-01 false Hearing. 6.54 Section 6.54 Labor Office of the Secretary of... Length Proceedings § 6.54 Hearing. (a) Except as provided in § 6.53(c) of this title, the hearing shall..., including the presentation of additional evidence, shall be considered at the hearing. There shall be...

  18. 49 CFR 240.409 - Hearings.

    Code of Federal Regulations, 2010 CFR

    2010-10-01

    ... 49 Transportation 4 2010-10-01 2010-10-01 false Hearings. 240.409 Section 240.409 Transportation... Hearings. (a) An administrative hearing for a locomotive engineer qualification petition shall be conducted... conduct of the hearing for the purpose of achieving a prompt and fair determination of all material...

  19. 38 CFR 18.9 - Hearings.

    Code of Federal Regulations, 2011 CFR

    2011-07-01

    ... 38 Pensions, Bonuses, and Veterans' Relief 2 2011-07-01 2011-07-01 false Hearings. 18.9 Section 18... THE CIVIL RIGHTS ACT OF 1964 General § 18.9 Hearings. (a) Opportunity for hearing. Whenever an opportunity for a hearing is required by § 18.8(c), reasonable notice shall be given by registered...

  20. 10 CFR 824.8 - Hearing.

    Code of Federal Regulations, 2011 CFR

    2011-01-01

    ... 10 Energy 4 2011-01-01 2011-01-01 false Hearing. 824.8 Section 824.8 Energy DEPARTMENT OF ENERGY... Hearing. (a) Any person who receives a final notice of violation under § 824.7 may request a hearing... hearing to the Director within 30 calendar days of receipt of the final notice of violation. (b)...

  1. 15 CFR 904.103 - Hearing.

    Code of Federal Regulations, 2011 CFR

    2011-01-01

    ... 15 Commerce and Foreign Trade 3 2011-01-01 2011-01-01 false Hearing. 904.103 Section 904.103 Commerce and Foreign Trade Regulations Relating to Commerce and Foreign Trade (Continued) NATIONAL OCEANIC... Penalties § 904.103 Hearing. (a) Any hearing request under § 904.102(a)(3) is governed by the hearing...

  2. 5 CFR 1639.23 - Hearing.

    Code of Federal Regulations, 2011 CFR

    2011-01-01

    ... 5 Administrative Personnel 3 2011-01-01 2011-01-01 false Hearing. 1639.23 Section 1639.23... Hearing. (a) Request for hearing. Except as provided in paragraph (b) of this section, an employee who desires a hearing concerning the existence or amount of the debt or the proposed offset schedule must...

  3. 22 CFR 141.8 - Hearings.

    Code of Federal Regulations, 2010 CFR

    2010-04-01

    ... 22 Foreign Relations 1 2010-04-01 2010-04-01 false Hearings. 141.8 Section 141.8 Foreign Relations...-EFFECTUATION OF TITLE VI OF THE CIVIL RIGHTS ACT OF 1964 § 141.8 Hearings. (a) Opportunity for hearing. Whenever an opportunity for a hearing is required by § 141.7(c), reasonable notice shall be given...

  4. 29 CFR 31.9 - Hearings.

    Code of Federal Regulations, 2011 CFR

    2011-07-01

    ... 29 Labor 1 2011-07-01 2011-07-01 false Hearings. 31.9 Section 31.9 Labor Office of the Secretary... TITLE VI OF THE CIVIL RIGHTS ACT OF 1964 § 31.9 Hearings. (a) Opportunity for hearing. Whenever an opportunity for a hearing is required by § 31.8(b), reasonable notice shall be given by registered...

  5. 15 CFR 280.214 - Hearings.

    Code of Federal Regulations, 2011 CFR

    2011-01-01

    ... 15 Commerce and Foreign Trade 1 2011-01-01 2011-01-01 false Hearings. 280.214 Section 280.214... Enforcement § 280.214 Hearings. (a) Scheduling. The administrative law judge, by agreement with the parties or upon notice to all parties of not less than 30 days, will schedule a hearing. All hearings will be...

  6. 10 CFR 2.1405 - Hearing.

    Code of Federal Regulations, 2010 CFR

    2010-01-01

    ... 10 Energy 1 2010-01-01 2010-01-01 false Hearing. 2.1405 Section 2.1405 Energy NUCLEAR REGULATORY... with Oral Hearings § 2.1405 Hearing. (a) No later than twenty (20) days after the conclusion of the prehearing conference, the presiding officer shall hold a hearing on any contention that remains in...

  7. 45 CFR 1110.9 - Hearings.

    Code of Federal Regulations, 2010 CFR

    2010-10-01

    ... 45 Public Welfare 3 2010-10-01 2010-10-01 false Hearings. 1110.9 Section 1110.9 Public Welfare... NONDISCRIMINATION IN FEDERALLY ASSISTED PROGRAMS § 1110.9 Hearings. (a) Opportunity for hearing. Whenever an opportunity for a hearing is required by § 1110.8(c), reasonable notice shall be given by registered...

  8. 29 CFR 4221.6 - Hearing.

    Code of Federal Regulations, 2010 CFR

    2010-07-01

    ... 29 Labor 9 2010-07-01 2010-07-01 false Hearing. 4221.6 Section 4221.6 Labor Regulations Relating... PLANS ARBITRATION OF DISPUTES IN MULTIEMPLOYER PLANS § 4221.6 Hearing. (a) Time and place of hearing established. Unless the parties agree to proceed without a hearing as provided in § 4221.5(c), the parties...

  9. 15 CFR 719.14 - Hearings.

    Code of Federal Regulations, 2011 CFR

    2011-01-01

    ... 15 Commerce and Foreign Trade 2 2011-01-01 2011-01-01 false Hearings. 719.14 Section 719.14... Hearings. (a) Scheduling. Upon receipt of a written and dated request for a hearing, the ALJ shall, by agreement with all the parties or upon notice to all parties of at least 30 days, schedule a hearing....

  10. 18 CFR 705.9 - Hearings.

    Code of Federal Regulations, 2011 CFR

    2011-04-01

    ... 18 Conservation of Power and Water Resources 2 2011-04-01 2011-04-01 false Hearings. 705.9 Section... ASSISTED PROGRAMS-EFFECTUATION OF TITLE VI OF THE CIVIL RIGHTS ACT OF 1964 § 705.9 Hearings. (a) Opportunity for hearing. Whenever an opportunity for a hearing is required by § 705.8(c), reasonable...

  11. 38 CFR 18.9 - Hearings.

    Code of Federal Regulations, 2010 CFR

    2010-07-01

    ... 38 Pensions, Bonuses, and Veterans' Relief 2 2010-07-01 2010-07-01 false Hearings. 18.9 Section 18... THE CIVIL RIGHTS ACT OF 1964 General § 18.9 Hearings. (a) Opportunity for hearing. Whenever an opportunity for a hearing is required by § 18.8(c), reasonable notice shall be given by registered...

  12. 29 CFR 1905.26 - Hearings.

    Code of Federal Regulations, 2010 CFR

    2010-07-01

    ... 29 Labor 5 2010-07-01 2010-07-01 false Hearings. 1905.26 Section 1905.26 Labor Regulations... OCCUPATIONAL SAFETY AND HEALTH ACT OF 1970 Hearings § 1905.26 Hearings. (a) Order of proceeding. Except as may be ordered otherwise by the presiding hearing examiner, the party applicant for relief shall...

  13. 5 CFR 1639.23 - Hearing.

    Code of Federal Regulations, 2010 CFR

    2010-01-01

    ... 5 Administrative Personnel 3 2010-01-01 2010-01-01 false Hearing. 1639.23 Section 1639.23... Hearing. (a) Request for hearing. Except as provided in paragraph (b) of this section, an employee who desires a hearing concerning the existence or amount of the debt or the proposed offset schedule must...

  14. 34 CFR 668.116 - Hearing.

    Code of Federal Regulations, 2011 CFR

    2011-07-01

    ... 34 Education 3 2011-07-01 2011-07-01 false Hearing. 668.116 Section 668.116 Education Regulations... Program Review Determinations § 668.116 Hearing. (a) A hearing is a process conducted by the hearing official whereby an orderly presentation of arguments and evidence is made by the parties. (b) The...

  15. 29 CFR 2200.209 - Hearing.

    Code of Federal Regulations, 2010 CFR

    2010-07-01

    ... 29 Labor 9 2010-07-01 2010-07-01 false Hearing. 2200.209 Section 2200.209 Labor Regulations... Simplified Proceedings § 2200.209 Hearing. (a) Procedures. As soon as practicable after the conclusion of the pre-hearing conference, the Judge will hold a hearing on any issue that remains in dispute....

  16. 20 CFR 901.44 - Hearings.

    Code of Federal Regulations, 2011 CFR

    2011-04-01

    ... 20 Employees' Benefits 3 2011-04-01 2011-04-01 false Hearings. 901.44 Section 901.44 Employees... § 901.44 Hearings. (a) In general. The Administrative Law Judge shall preside at the hearing on a complaint for the suspension or termination of an enrolled actuary. Hearings shall be...

  17. 18 CFR 156.10 - Hearings.

    Code of Federal Regulations, 2010 CFR

    2010-04-01

    ... 18 Conservation of Power and Water Resources 1 2010-04-01 2010-04-01 false Hearings. 156.10... GAS ACT § 156.10 Hearings. The Commission will schedule each application for public hearing at the... protests or petitions to intervene, issue the requested order without hearing....

  18. 45 CFR 80.9 - Hearings.

    Code of Federal Regulations, 2010 CFR

    2010-10-01

    ... 45 Public Welfare 1 2010-10-01 2010-10-01 false Hearings. 80.9 Section 80.9 Public Welfare... CIVIL RIGHTS ACT OF 1964 § 80.9 Hearings. (a) Opportunity for hearing. Whenever an opportunity for a hearing is required by § 80.8(c), reasonable notice shall be given by registered or certified mail,...

  19. 39 CFR 3001.30 - Hearings.

    Code of Federal Regulations, 2011 CFR

    2011-07-01

    ... 39 Postal Service 1 2011-07-01 2011-07-01 false Hearings. 3001.30 Section 3001.30 Postal Service... § 3001.30 Hearings. (a) How initiated. Hearings for the purpose of taking evidence shall be initiated by... hearings shall be held before the Commission sitting en banc, or a duly designated presiding officer....

  20. 7 CFR 15.9 - Hearings.

    Code of Federal Regulations, 2010 CFR

    2010-01-01

    ... 7 Agriculture 1 2010-01-01 2010-01-01 false Hearings. 15.9 Section 15.9 Agriculture Office of the... Department of Agriculture-Effectuation of Title VI of the Civil Rights Act of 1964 § 15.9 Hearings. (a) Opportunity for hearing. Whenever an opportunity for a hearing is required under the regulations in this...

  1. 5 CFR 900.409 - Hearings.

    Code of Federal Regulations, 2010 CFR

    2010-01-01

    ... 5 Administrative Personnel 2 2010-01-01 2010-01-01 false Hearings. 900.409 Section 900.409... Management-Effectuation of Title VI of the Civil Rights Act of 1964 § 900.409 Hearings. (a) Opportunity for hearing. When an opportunity for a hearing is required by § 900.408(c), reasonable notice shall be...

  2. 13 CFR 117.16 - Hearings.

    Code of Federal Regulations, 2010 CFR

    2010-01-01

    ... conformity with the Administrative Procedure Act and 13 CFR part 134. Such rules of procedure should be... 13 Business Credit and Assistance 1 2010-01-01 2010-01-01 false Hearings. 117.16 Section 117.16... Hearings. (a) Opportunity for hearing. Whenever an opportunity for a hearing is required by §...

  3. 75 FR 12584 - Sunshine Act; Public Hearing

    Federal Register 2010, 2011, 2012, 2013, 2014

    2010-03-16

    ... CORPORATION Sunshine Act; Public Hearing March 17, 2010. OPIC's Sunshine Act notice of its Public Hearing in... record; therefore, OPIC's public hearing scheduled for 3 p.m., March 17, 2010 in conjunction with OPIC's...: Information on the hearing cancellation may be obtained from Connie M. Downs at (202) 336-8438, via...

  4. 12 CFR 308.142 - Hearing.

    Code of Federal Regulations, 2010 CFR

    2010-01-01

    ... 12 Banks and Banking 4 2010-01-01 2010-01-01 false Hearing. 308.142 Section 308.142 Banks and... Exchange Act of 1934 § 308.142 Hearing. (a) Proceedings are informal. Formal rules of evidence, the... Local Rules shall not apply to hearings under this subpart. (b) Hearing Procedure. (1) Parties to...

  5. 18 CFR 705.9 - Hearings.

    Code of Federal Regulations, 2010 CFR

    2010-04-01

    ... 18 Conservation of Power and Water Resources 2 2010-04-01 2010-04-01 false Hearings. 705.9 Section... ASSISTED PROGRAMS-EFFECTUATION OF TITLE VI OF THE CIVIL RIGHTS ACT OF 1964 § 705.9 Hearings. (a) Opportunity for hearing. Whenever an opportunity for a hearing is required by § 705.8(c), reasonable...

  6. 12 CFR 268.108 - Hearings.

    Code of Federal Regulations, 2010 CFR

    2010-01-01

    ... 12 Banks and Banking 3 2010-01-01 2010-01-01 false Hearings. 268.108 Section 268.108 Banks and... REGARDING EQUAL OPPORTUNITY Board Program To Promote Equal Opportunity § 268.108 Hearings. (a) When a complainant requests a hearing, the Commission shall appoint an administrative judge to conduct a hearing...

  7. 76 FR 66126 - Notice of Public Hearing

    Federal Register 2010, 2011, 2012, 2013, 2014

    2011-10-25

    ... Federal Railroad Administration Notice of Public Hearing The Central Oregon and Pacific Railroad (CORP... the carrier's proposal and the available facts, FRA has determined that a public hearing is necessary... participate in a public hearing on December 1, 2011. The hearing will be conducted at the Douglas...

  8. 75 FR 67145 - Sunshine Act: Public Hearing

    Federal Register 2010, 2011, 2012, 2013, 2014

    2010-11-01

    ... CORPORATION Sunshine Act: Public Hearing TIME AND DATE: 2 p.m., Wednesday, November 24, 2010. PLACE: Offices...: Hearing open to the Public at 2 p.m. PURPOSE: Public Hearing in conjunction with each meeting of OPIC's... the Corporation. pROCEDURES: Individuals wishing to address the hearing orally must provide...

  9. 12 CFR 308.142 - Hearing.

    Code of Federal Regulations, 2011 CFR

    2011-01-01

    ... 12 Banks and Banking 4 2011-01-01 2011-01-01 false Hearing. 308.142 Section 308.142 Banks and... Exchange Act of 1934 § 308.142 Hearing. (a) Proceedings are informal. Formal rules of evidence, the... Local Rules shall not apply to hearings under this subpart. (b) Hearing Procedure. (1) Parties to...

  10. 7 CFR 900.115 - Hearing.

    Code of Federal Regulations, 2010 CFR

    2010-01-01

    ... 7 Agriculture 8 2010-01-01 2010-01-01 false Hearing. 900.115 Section 900.115 Agriculture... Hearing. (a) The arbitrator shall have full discretion to conduct the hearing in such manner as will, in..., and other experts. (h) When more than two arbitrators are designated to hear a dispute, and...

  11. 5 CFR 831.1106 - Hearing.

    Code of Federal Regulations, 2010 CFR

    2010-01-01

    ... 5 Administrative Personnel 2 2010-01-01 2010-01-01 false Hearing. 831.1106 Section 831.1106...) RETIREMENT Prohibition on Payments of Annuities § 831.1106 Hearing. (a) OPM's hearing examiner shall preside at any hearing held pursuant to this subpart, unless OPM designates another presiding officer....

  12. 34 CFR 100.9 - Hearings.

    Code of Federal Regulations, 2010 CFR

    2010-07-01

    ... 34 Education 1 2010-07-01 2010-07-01 false Hearings. 100.9 Section 100.9 Education Regulations of... TITLE VI OF THE CIVIL RIGHTS ACT OF 1964 § 100.9 Hearings. (a) Opportunity for hearing. Whenever an opportunity for a hearing is required by § 100.8(c), reasonable notice shall be given by registered...

  13. 34 CFR 100.9 - Hearings.

    Code of Federal Regulations, 2011 CFR

    2011-07-01

    ... 34 Education 1 2011-07-01 2011-07-01 false Hearings. 100.9 Section 100.9 Education Regulations of... TITLE VI OF THE CIVIL RIGHTS ACT OF 1964 § 100.9 Hearings. (a) Opportunity for hearing. Whenever an opportunity for a hearing is required by § 100.8(c), reasonable notice shall be given by registered...

  14. 18 CFR 1307.11 - Hearings.

    Code of Federal Regulations, 2011 CFR

    2011-04-01

    ... 18 Conservation of Power and Water Resources 2 2011-04-01 2011-04-01 false Hearings. 1307.11... WITH RESPECT TO HANDICAP § 1307.11 Hearings. (a) Opportunity for hearing. Whenever an opportunity for a hearing is required by § 1307.10, reasonable notice shall be given by registered or certified mail,...

  15. 39 CFR 775.14 - Hearings.

    Code of Federal Regulations, 2011 CFR

    2011-07-01

    ... 39 Postal Service 1 2011-07-01 2011-07-01 false Hearings. 775.14 Section 775.14 Postal Service....14 Hearings. (a) Public hearings must be held whenever there is: (1) Substantial environmental controversy concerning a proposed action and a request for a hearing by any responsible individual...

  16. 45 CFR 1606.8 - Hearing.

    Code of Federal Regulations, 2010 CFR

    2010-10-01

    ... 45 Public Welfare 4 2010-10-01 2010-10-01 false Hearing. 1606.8 Section 1606.8 Public Welfare... PROCEDURES; RECOMPETITION § 1606.8 Hearing. (a) The recipient may make written request for a hearing within... days after receipt of a request for a hearing, the Corporation shall notify the recipient in writing...

  17. 19 CFR 351.310 - Hearings.

    Code of Federal Regulations, 2010 CFR

    2010-04-01

    ... 19 Customs Duties 3 2010-04-01 2010-04-01 false Hearings. 351.310 Section 351.310 Customs Duties... Argument § 351.310 Hearings. (a) Introduction. This section sets forth the procedures for requesting a hearing, indicates that the Secretary may consolidate hearings, and explains when the Secretary may...

  18. 22 CFR 209.9 - Hearings.

    Code of Federal Regulations, 2011 CFR

    2011-04-01

    ... 22 Foreign Relations 1 2011-04-01 2011-04-01 false Hearings. 209.9 Section 209.9 Foreign Relations... INTERNATIONAL DEVELOPMENT-EFFECTUATION OF TITLE VI OF THE CIVIL RIGHTS ACT OF 1964 § 209.9 Hearings. (a) Opportunity for hearing. Whenever an opportunity for a hearing is required by § 209.8(c), reasonable...

  19. 41 CFR 50-203.8 - Hearing.

    Code of Federal Regulations, 2011 CFR

    2011-07-01

    ... 41 Public Contracts and Property Management 1 2011-07-01 2009-07-01 true Hearing. 50-203.8 Section...-Healey Public Contracts Act § 50-203.8 Hearing. (a) The hearing for the purpose of taking evidence upon a... law judge may be designated to take his place. Such hearings shall be open to the public...

  20. 45 CFR 509.6 - Hearings.

    Code of Federal Regulations, 2010 CFR

    2010-10-01

    ... 45 Public Welfare 3 2010-10-01 2010-10-01 false Hearings. 509.6 Section 509.6 Public Welfare... Hearings. (a) Hearings, whether upon the Commission's own motion or upon request of claimant, will be held upon not less than fifteen days' notice of the time and place thereof. (b) The hearings will be open...

  1. 45 CFR 509.6 - Hearings.

    Code of Federal Regulations, 2011 CFR

    2011-10-01

    ... 45 Public Welfare 3 2011-10-01 2011-10-01 false Hearings. 509.6 Section 509.6 Public Welfare... Hearings. (a) Hearings, whether upon the Commission's own motion or upon request of claimant, will be held upon not less than fifteen days' notice of the time and place thereof. (b) The hearings will be open...

  2. 5 CFR 1215.5 - Hearing.

    Code of Federal Regulations, 2011 CFR

    2011-01-01

    ... Federal Claims Collection Standards 4 CFR 102.3(c). The burden shall be on the employee to demonstrate... 5 Administrative Personnel 3 2011-01-01 2011-01-01 false Hearing. 1215.5 Section 1215.5... § 1215.5 Hearing. (a) Request for hearing. (1) An employee must file a petition for a hearing...

  3. 45 CFR 1606.8 - Hearing.

    Code of Federal Regulations, 2011 CFR

    2011-10-01

    ... 45 Public Welfare 4 2011-10-01 2011-10-01 false Hearing. 1606.8 Section 1606.8 Public Welfare... PROCEDURES; RECOMPETITION § 1606.8 Hearing. (a) The recipient may make written request for a hearing within... days after receipt of a request for a hearing, the Corporation shall notify the recipient in writing...

  4. 7 CFR 900.115 - Hearing.

    Code of Federal Regulations, 2011 CFR

    2011-01-01

    ... 7 Agriculture 8 2011-01-01 2011-01-01 false Hearing. 900.115 Section 900.115 Agriculture... Hearing. (a) The arbitrator shall have full discretion to conduct the hearing in such manner as will, in..., and other experts. (h) When more than two arbitrators are designated to hear a dispute, and...

  5. 22 CFR 141.8 - Hearings.

    Code of Federal Regulations, 2011 CFR

    2011-04-01

    ... 22 Foreign Relations 1 2011-04-01 2011-04-01 false Hearings. 141.8 Section 141.8 Foreign Relations...-EFFECTUATION OF TITLE VI OF THE CIVIL RIGHTS ACT OF 1964 § 141.8 Hearings. (a) Opportunity for hearing. Whenever an opportunity for a hearing is required by § 141.7(c), reasonable notice shall be given...

  6. 49 CFR 209.321 - Hearing.

    Code of Federal Regulations, 2010 CFR

    2010-10-01

    ... 49 Transportation 4 2010-10-01 2010-10-01 false Hearing. 209.321 Section 209.321 Transportation... TRANSPORTATION RAILROAD SAFETY ENFORCEMENT PROCEDURES Disqualification Procedures § 209.321 Hearing. (a) Upon receipt of a hearing request complying with § 209.311, an administrative hearing for review of a notice...

  7. 15 CFR 766.13 - Hearings.

    Code of Federal Regulations, 2011 CFR

    2011-01-01

    ... 15 Commerce and Foreign Trade 2 2011-01-01 2011-01-01 false Hearings. 766.13 Section 766.13... PROCEEDINGS § 766.13 Hearings. (a) Scheduling. The administrative law judge, by agreement with the parties or upon notice to all parties of not less than 30 days, will schedule a hearing. All hearings will be...

  8. 15 CFR 280.214 - Hearings.

    Code of Federal Regulations, 2010 CFR

    2010-01-01

    ... 15 Commerce and Foreign Trade 1 2010-01-01 2010-01-01 false Hearings. 280.214 Section 280.214... Enforcement § 280.214 Hearings. (a) Scheduling. The administrative law judge, by agreement with the parties or upon notice to all parties of not less than 30 days, will schedule a hearing. All hearings will be...

  9. 39 CFR 953.5 - Hearings.

    Code of Federal Regulations, 2011 CFR

    2011-07-01

    ... 39 Postal Service 1 2011-07-01 2011-07-01 false Hearings. 953.5 Section 953.5 Postal Service... Hearings. (a) In general, admissibility of evidence at hearings conducted under this part hinges on... the rulings of the presiding officer made during the course of the hearing are unnecessary. For...

  10. 45 CFR 80.9 - Hearings.

    Code of Federal Regulations, 2011 CFR

    2011-10-01

    ... 45 Public Welfare 1 2011-10-01 2011-10-01 false Hearings. 80.9 Section 80.9 Public Welfare... CIVIL RIGHTS ACT OF 1964 § 80.9 Hearings. (a) Opportunity for hearing. Whenever an opportunity for a hearing is required by § 80.8(c), reasonable notice shall be given by registered or certified mail,...

  11. 29 CFR 30.16 - Hearings.

    Code of Federal Regulations, 2011 CFR

    2011-07-01

    ... 29 Labor 1 2011-07-01 2011-07-01 false Hearings. 30.16 Section 30.16 Labor Office of the Secretary of Labor EQUAL EMPLOYMENT OPPORTUNITY IN APPRENTICESHIP AND TRAINING § 30.16 Hearings. (a) Within 10 days after receiving a request for a hearing, the Secretary shall designate a hearing officer....

  12. 19 CFR 207.66 - Hearing.

    Code of Federal Regulations, 2010 CFR

    2010-04-01

    ... 19 Customs Duties 3 2010-04-01 2010-04-01 false Hearing. 207.66 Section 207.66 Customs Duties... EXPORTS TO THE UNITED STATES Five-Year Reviews § 207.66 Hearing. (a) In general. The Commission shall hold a hearing in each full review. The date of the hearing shall be specified in the scheduling...

  13. 5 CFR 1215.5 - Hearing.

    Code of Federal Regulations, 2010 CFR

    2010-01-01

    ... Federal Claims Collection Standards 4 CFR 102.3(c). The burden shall be on the employee to demonstrate... 5 Administrative Personnel 3 2010-01-01 2010-01-01 false Hearing. 1215.5 Section 1215.5... § 1215.5 Hearing. (a) Request for hearing. (1) An employee must file a petition for a hearing...

  14. 19 CFR 207.112 - Hearings.

    Code of Federal Regulations, 2010 CFR

    2010-04-01

    ... 19 Customs Duties 3 2010-04-01 2010-04-01 false Hearings. 207.112 Section 207.112 Customs Duties... and Committee Proceedings § 207.112 Hearings. (a) Purpose of and scheduling of hearings. An opportunity for a hearing before an administrative law judge shall be provided for each action initiated...

  15. 29 CFR 301.7 - Hearings.

    Code of Federal Regulations, 2010 CFR

    2010-07-01

    ... 29 Labor 2 2010-07-01 2010-07-01 false Hearings. 301.7 Section 301.7 Labor Regulations Relating to Labor NATIONAL RAILROAD ADJUSTMENT BOARD RULES OF PROCEDURE § 301.7 Hearings. (a) Oral hearings will be... and date of the hearing. (b) The parties are, however, charged with the duty and responsibility...

  16. 45 CFR 96.51 - Hearings.

    Code of Federal Regulations, 2011 CFR

    2011-10-01

    ... 45 Public Welfare 1 2011-10-01 2011-10-01 false Hearings. 96.51 Section 96.51 Public Welfare DEPARTMENT OF HEALTH AND HUMAN SERVICES GENERAL ADMINISTRATION BLOCK GRANTS Enforcement § 96.51 Hearings. (a... notice of the order and an opportunity for a hearing. Opportunity for a hearing will not be...

  17. 19 CFR 354.12 - Hearing.

    Code of Federal Regulations, 2010 CFR

    2010-04-01

    ... 19 Customs Duties 3 2010-04-01 2010-04-01 false Hearing. 354.12 Section 354.12 Customs Duties... ANTIDUMPING OR COUNTERVAILING DUTY ADMINISTRATIVE PROTECTIVE ORDER § 354.12 Hearing. (a) Scheduling of hearing. The presiding official will schedule the hearing at a reasonable time, date, and place, which will...

  18. 22 CFR 18.15 - Hearings.

    Code of Federal Regulations, 2011 CFR

    2011-04-01

    ... 22 Foreign Relations 1 2011-04-01 2011-04-01 false Hearings. 18.15 Section 18.15 Foreign Relations... Enforcement Proceedings § 18.15 Hearings. Hearings shall be stenographically recorded and transcribed and the testimony of witnesses shall be taken under oath or affirmation. Hearings will be closed unless an...

  19. 45 CFR 1110.9 - Hearings.

    Code of Federal Regulations, 2011 CFR

    2011-10-01

    ... 45 Public Welfare 3 2011-10-01 2011-10-01 false Hearings. 1110.9 Section 1110.9 Public Welfare... NONDISCRIMINATION IN FEDERALLY ASSISTED PROGRAMS § 1110.9 Hearings. (a) Opportunity for hearing. Whenever an opportunity for a hearing is required by § 1110.8(c), reasonable notice shall be given by registered...

  20. 47 CFR 1.1928 - Hearing.

    Code of Federal Regulations, 2010 CFR

    2010-10-01

    ... 47 Telecommunication 1 2010-10-01 2010-10-01 false Hearing. 1.1928 Section 1.1928... United States Salary Offset-Individual Debt § 1.1928 Hearing. (a) Petition for hearing. (1) An employee may request a hearing by filing a written petition with the Managing Director of the Commission,...

  1. 22 CFR 18.15 - Hearings.

    Code of Federal Regulations, 2010 CFR

    2010-04-01

    ... 22 Foreign Relations 1 2010-04-01 2010-04-01 false Hearings. 18.15 Section 18.15 Foreign Relations... Enforcement Proceedings § 18.15 Hearings. Hearings shall be stenographically recorded and transcribed and the testimony of witnesses shall be taken under oath or affirmation. Hearings will be closed unless an...

  2. 49 CFR 386.56 - Hearings.

    Code of Federal Regulations, 2011 CFR

    2011-10-01

    ... 49 Transportation 5 2011-10-01 2011-10-01 false Hearings. 386.56 Section 386.56 Transportation... and Hearings § 386.56 Hearings. (a) As soon as practicable after his/her appointment, the administrative law judge shall issue an order setting the date, time, and place for the hearing. The order...

  3. 10 Ways to Identify Hearing Loss

    MedlinePlus

    ... of this page please turn JavaScript on. Feature: Hearing Loss 10 Ways to Identify Hearing Loss Past Issues / Spring 2015 Table of Contents If ... gov Internet: www.nidcd.nih.gov Read More "Hearing Loss" Articles Managing Hearing Loss / Symptoms, Devices, Prevention & Research / ...

  4. 39 CFR 955.9 - Hearing election.

    Code of Federal Regulations, 2011 CFR

    2011-07-01

    ... 39 Postal Service 1 2011-07-01 2011-07-01 false Hearing election. 955.9 Section 955.9 Postal... CONTRACT APPEALS § 955.9 Hearing election. Link to an amendment published at 76 FR 37660, June 28, 2011. As... hearing as prescribed in § 955.12. If a hearing is elected, the election should state where and when...

  5. 45 CFR 33.6 - Hearings.

    Code of Federal Regulations, 2010 CFR

    2010-10-01

    ... 45 Public Welfare 1 2010-10-01 2010-10-01 false Hearings. 33.6 Section 33.6 Public Welfare DEPARTMENT OF HEALTH AND HUMAN SERVICES GENERAL ADMINISTRATION SALARY OFFSET § 33.6 Hearings. (a) Petitions for hearing. (1) To request a hearing concerning the existence or amount of the debt or the offset schedule established by the Department,...

  6. 17 CFR 10.64 - Public hearings.

    Code of Federal Regulations, 2012 CFR

    2012-04-01

    ... 17 Commodity and Securities Exchanges 1 2012-04-01 2012-04-01 false Public hearings. 10.64 Section 10.64 Commodity and Securities Exchanges COMMODITY FUTURES TRADING COMMISSION RULES OF PRACTICE Hearings § 10.64 Public hearings. All hearings shall be public, except that upon application of a respondent or affected witness the Administrative...

  7. 17 CFR 10.64 - Public hearings.

    Code of Federal Regulations, 2011 CFR

    2011-04-01

    ... 17 Commodity and Securities Exchanges 1 2011-04-01 2011-04-01 false Public hearings. 10.64 Section 10.64 Commodity and Securities Exchanges COMMODITY FUTURES TRADING COMMISSION RULES OF PRACTICE Hearings § 10.64 Public hearings. All hearings shall be public, except that upon application of a respondent or affected witness the Administrative...

  8. 17 CFR 10.64 - Public hearings.

    Code of Federal Regulations, 2014 CFR

    2014-04-01

    ... 17 Commodity and Securities Exchanges 1 2014-04-01 2014-04-01 false Public hearings. 10.64 Section 10.64 Commodity and Securities Exchanges COMMODITY FUTURES TRADING COMMISSION RULES OF PRACTICE Hearings § 10.64 Public hearings. All hearings shall be public, except that upon application of a respondent or affected witness the Administrative...

  9. 5 CFR 1215.5 - Hearing.

    Code of Federal Regulations, 2014 CFR

    2014-01-01

    ... Federal Claims Collection Standards 4 CFR 102.3(c). The burden shall be on the employee to demonstrate... 5 Administrative Personnel 3 2014-01-01 2014-01-01 false Hearing. 1215.5 Section 1215.5... § 1215.5 Hearing. (a) Request for hearing. (1) An employee must file a petition for a hearing...

  10. 5 CFR 1201.52 - Public hearings.

    Code of Federal Regulations, 2010 CFR

    2010-01-01

    ... 5 Administrative Personnel 3 2010-01-01 2010-01-01 false Public hearings. 1201.52 Section 1201.52 Administrative Personnel MERIT SYSTEMS PROTECTION BOARD ORGANIZATION AND PROCEDURES PRACTICES AND PROCEDURES Procedures for Appellate Cases Hearings § 1201.52 Public hearings. Hearings are open to the public. The...

  11. 15 CFR 705.8 - Public hearings.

    Code of Federal Regulations, 2010 CFR

    2010-01-01

    ... additional information or to request time to speak at the hearing shall also be included. Public hearings may... 15 Commerce and Foreign Trade 2 2010-01-01 2010-01-01 false Public hearings. 705.8 Section 705.8... IMPORTED ARTICLES ON THE NATIONAL SECURITY § 705.8 Public hearings. (a) If it is deemed appropriate by...

  12. 21 CFR 1313.51 - Hearings generally.

    Code of Federal Regulations, 2012 CFR

    2012-04-01

    .... 100-690), by 21 CFR 1313.52-1313.57, and by the procedures for administrative hearings under the... 21 Food and Drugs 9 2012-04-01 2012-04-01 false Hearings generally. 1313.51 Section 1313.51 Food... AND LIST II CHEMICALS Hearings § 1313.51 Hearings generally. In any case where a regulated...

  13. 44 CFR 68.4 - Hearing board.

    Code of Federal Regulations, 2010 CFR

    2010-10-01

    ... 44 Emergency Management and Assistance 1 2010-10-01 2010-10-01 false Hearing board. 68.4 Section 68.4 Emergency Management and Assistance FEDERAL EMERGENCY MANAGEMENT AGENCY, DEPARTMENT OF HOMELAND... § 68.4 Hearing board. (a) Each hearing shall be conducted by a three member hearing board...

  14. 7 CFR 247.33 - Fair hearings.

    Code of Federal Regulations, 2012 CFR

    2012-01-01

    ... 7 Agriculture 4 2012-01-01 2012-01-01 false Fair hearings. 247.33 Section 247.33 Agriculture Regulations of the Department of Agriculture (Continued) FOOD AND NUTRITION SERVICE, DEPARTMENT OF AGRICULTURE CHILD NUTRITION PROGRAMS COMMODITY SUPPLEMENTAL FOOD PROGRAM § 247.33 Fair hearings. (a) What is a fair hearing? A fair hearing is a...

  15. 7 CFR 247.33 - Fair hearings.

    Code of Federal Regulations, 2014 CFR

    2014-01-01

    ... 7 Agriculture 4 2014-01-01 2014-01-01 false Fair hearings. 247.33 Section 247.33 Agriculture Regulations of the Department of Agriculture (Continued) FOOD AND NUTRITION SERVICE, DEPARTMENT OF AGRICULTURE CHILD NUTRITION PROGRAMS COMMODITY SUPPLEMENTAL FOOD PROGRAM § 247.33 Fair hearings. (a) What is a fair hearing? A fair hearing is a...

  16. 42 CFR 423.1040 - Joint hearings.

    Code of Federal Regulations, 2014 CFR

    2014-10-01

    ... 42 Public Health 3 2014-10-01 2014-10-01 false Joint hearings. 423.1040 Section 423.1040 Public... Penalties § 423.1040 Joint hearings. When two or more affected parties have requested hearings and the same... and place for the prehearing conference or hearing and conduct all proceedings jointly. If...

  17. 42 CFR 423.1040 - Joint hearings.

    Code of Federal Regulations, 2013 CFR

    2013-10-01

    ... 42 Public Health 3 2013-10-01 2013-10-01 false Joint hearings. 423.1040 Section 423.1040 Public... Penalties § 423.1040 Joint hearings. When two or more affected parties have requested hearings and the same... and place for the prehearing conference or hearing and conduct all proceedings jointly. If...

  18. 42 CFR 423.1040 - Joint hearings.

    Code of Federal Regulations, 2012 CFR

    2012-10-01

    ... 42 Public Health 3 2012-10-01 2012-10-01 false Joint hearings. 423.1040 Section 423.1040 Public... Penalties § 423.1040 Joint hearings. When two or more affected parties have requested hearings and the same... and place for the prehearing conference or hearing and conduct all proceedings jointly. If...

  19. 17 CFR 204.64 - Hearing.

    Code of Federal Regulations, 2010 CFR

    2010-04-01

    ... Chief Administrative Law Judge shall select, pursuant to 17 CFR 200.30-10, the administrative law judge... decision by a hearing official pursuant to 17 CFR 201.431(c), a decision by a hearing official shall become... Administrative Wage Garnishment § 204.64 Hearing. (a) Request for hearing. The Commission will order a...

  20. 17 CFR 204.64 - Hearing.

    Code of Federal Regulations, 2011 CFR

    2011-04-01

    ... Chief Administrative Law Judge shall select, pursuant to 17 CFR 200.30-10, the administrative law judge... decision by a hearing official pursuant to 17 CFR 201.431(c), a decision by a hearing official shall become... Administrative Wage Garnishment § 204.64 Hearing. (a) Request for hearing. The Commission will order a...

  1. 12 CFR 908.22 - Public hearings.

    Code of Federal Regulations, 2011 CFR

    2011-01-01

    ... 12 Banks and Banking 7 2011-01-01 2011-01-01 false Public hearings. 908.22 Section 908.22 Banks... RULES OF PRACTICE AND PROCEDURE IN HEARINGS ON THE RECORD General Rules § 908.22 Public hearings. (a) General rule. All hearings shall be open to the public, unless the Finance Board, in its...

  2. 12 CFR 263.33 - Public hearings.

    Code of Federal Regulations, 2014 CFR

    2014-01-01

    ... 12 Banks and Banking 4 2014-01-01 2014-01-01 false Public hearings. 263.33 Section 263.33 Banks... (CONTINUED) RULES OF PRACTICE FOR HEARINGS Uniform Rules of Practice and Procedure § 263.33 Public hearings. (a) General rule. All hearings shall be open to the public, unless the Board, in the...

  3. 5 CFR 1201.52 - Public hearings.

    Code of Federal Regulations, 2011 CFR

    2011-01-01

    ... 5 Administrative Personnel 3 2011-01-01 2011-01-01 false Public hearings. 1201.52 Section 1201.52... Procedures for Appellate Cases Hearings § 1201.52 Public hearings. Hearings are open to the public. The judge... interests of the appellant, a witness, the public, or any other person affected by the proceeding. Any...

  4. 15 CFR 2006.7 - Public hearings.

    Code of Federal Regulations, 2010 CFR

    2010-01-01

    ... 15 Commerce and Foreign Trade 3 2010-01-01 2010-01-01 false Public hearings. 2006.7 Section 2006.7... 1974, AS AMENDED § 2006.7 Public hearings. (a) A public hearing for the purpose of receiving views on... response to issues raised in the petition, the Section 301 Committee shall hold a public hearing upon...

  5. 12 CFR 263.33 - Public hearings.

    Code of Federal Regulations, 2010 CFR

    2010-01-01

    ... 12 Banks and Banking 3 2010-01-01 2010-01-01 false Public hearings. 263.33 Section 263.33 Banks... OF PRACTICE FOR HEARINGS Uniform Rules of Practice and Procedure § 263.33 Public hearings. (a) General rule. All hearings shall be open to the public, unless the Board, in the Board's...

  6. 10 CFR 110.100 - Public hearings.

    Code of Federal Regulations, 2011 CFR

    2011-01-01

    ... 10 Energy 2 2011-01-01 2011-01-01 false Public hearings. 110.100 Section 110.100 Energy NUCLEAR REGULATORY COMMISSION (CONTINUED) EXPORT AND IMPORT OF NUCLEAR EQUIPMENT AND MATERIAL Hearings § 110.100 Public hearings. Hearings under this part will be public unless the Commission directs otherwise....

  7. 15 CFR 705.8 - Public hearings.

    Code of Federal Regulations, 2013 CFR

    2013-01-01

    ... 15 Commerce and Foreign Trade 2 2013-01-01 2013-01-01 false Public hearings. 705.8 Section 705.8... IMPORTED ARTICLES ON THE NATIONAL SECURITY § 705.8 Public hearings. (a) If it is deemed appropriate by the Department, public hearings may be held to elicit further information. (1) A notice of hearing shall...

  8. 15 CFR 2006.7 - Public hearings.

    Code of Federal Regulations, 2011 CFR

    2011-01-01

    ... 15 Commerce and Foreign Trade 3 2011-01-01 2011-01-01 false Public hearings. 2006.7 Section 2006.7... 1974, AS AMENDED § 2006.7 Public hearings. (a) A public hearing for the purpose of receiving views on... response to issues raised in the petition, the Section 301 Committee shall hold a public hearing upon...

  9. 49 CFR 520.26 - Public hearings.

    Code of Federal Regulations, 2013 CFR

    2013-10-01

    ... 49 Transportation 6 2013-10-01 2013-10-01 false Public hearings. 520.26 Section 520.26... Public hearings. (a) A public hearing on a proposed or ongoing action covered by a DEIS shall be held... Administrator for Planning and Evaluation, that a public hearing would be appropriate and in the public...

  10. 15 CFR 2006.7 - Public hearings.

    Code of Federal Regulations, 2013 CFR

    2013-01-01

    ... 15 Commerce and Foreign Trade 3 2013-01-01 2013-01-01 false Public hearings. 2006.7 Section 2006.7... 1974, AS AMENDED § 2006.7 Public hearings. (a) A public hearing for the purpose of receiving views on... response to issues raised in the petition, the Section 301 Committee shall hold a public hearing upon...

  11. 5 CFR 1201.52 - Public hearings.

    Code of Federal Regulations, 2012 CFR

    2012-01-01

    ... 5 Administrative Personnel 3 2012-01-01 2012-01-01 false Public hearings. 1201.52 Section 1201.52... Procedures for Appellate Cases Hearings § 1201.52 Public hearings. Hearings are open to the public. The judge... interests of the appellant, a witness, the public, or any other person affected by the proceeding. Any...

  12. 15 CFR 705.8 - Public hearings.

    Code of Federal Regulations, 2011 CFR

    2011-01-01

    ... 15 Commerce and Foreign Trade 2 2011-01-01 2011-01-01 false Public hearings. 705.8 Section 705.8... IMPORTED ARTICLES ON THE NATIONAL SECURITY § 705.8 Public hearings. (a) If it is deemed appropriate by the Department, public hearings may be held to elicit further information. (1) A notice of hearing shall...

  13. 49 CFR 557.7 - Public hearing.

    Code of Federal Regulations, 2013 CFR

    2013-10-01

    ... 49 Transportation 6 2013-10-01 2013-10-01 false Public hearing. 557.7 Section 557.7 Transportation..., DEPARTMENT OF TRANSPORTATION PETITIONS FOR HEARINGS ON NOTIFICATION AND REMEDY OF DEFECTS § 557.7 Public hearing. If the Administrator decides that a public hearing under this part is necessary, he issues...

  14. 19 CFR 206.5 - Public hearing.

    Code of Federal Regulations, 2012 CFR

    2012-04-01

    ... 19 Customs Duties 3 2012-04-01 2012-04-01 false Public hearing. 206.5 Section 206.5 Customs Duties... General § 206.5 Public hearing. (a) Investigations under subpart B of this part. A public hearing on the question of injury and a second public hearing on remedy (if necessary) will be held in connection...

  15. 4 CFR 28.57 - Public hearings.

    Code of Federal Regulations, 2011 CFR

    2011-01-01

    ... 4 Accounts 1 2011-01-01 2011-01-01 false Public hearings. 28.57 Section 28.57 Accounts GOVERNMENT... Hearings § 28.57 Public hearings. (a) Hearings shall be open to the public. However, the administrative... in the best interests of the petitioner, a witness, the public, or other affected persons. Any...

  16. 15 CFR 2006.7 - Public hearings.

    Code of Federal Regulations, 2012 CFR

    2012-01-01

    ... 15 Commerce and Foreign Trade 3 2012-01-01 2012-01-01 false Public hearings. 2006.7 Section 2006.7... 1974, AS AMENDED § 2006.7 Public hearings. (a) A public hearing for the purpose of receiving views on... response to issues raised in the petition, the Section 301 Committee shall hold a public hearing upon...

  17. 17 CFR 10.64 - Public hearings.

    Code of Federal Regulations, 2013 CFR

    2013-04-01

    ... 17 Commodity and Securities Exchanges 1 2013-04-01 2013-04-01 false Public hearings. 10.64 Section 10.64 Commodity and Securities Exchanges COMMODITY FUTURES TRADING COMMISSION RULES OF PRACTICE Hearings § 10.64 Public hearings. All hearings shall be public, except that upon application of...

  18. 15 CFR 705.8 - Public hearings.

    Code of Federal Regulations, 2012 CFR

    2012-01-01

    ... 15 Commerce and Foreign Trade 2 2012-01-01 2012-01-01 false Public hearings. 705.8 Section 705.8... IMPORTED ARTICLES ON THE NATIONAL SECURITY § 705.8 Public hearings. (a) If it is deemed appropriate by the Department, public hearings may be held to elicit further information. (1) A notice of hearing shall...

  19. 12 CFR 390.62 - Public hearings.

    Code of Federal Regulations, 2012 CFR

    2012-01-01

    ... 12 Banks and Banking 5 2012-01-01 2012-01-01 false Public hearings. 390.62 Section 390.62 Banks... Proceedings § 390.62 Public hearings. (a) General rule. All hearings shall be open to the public, unless the FDIC, in its discretion, determines that holding an open hearing would be contrary to the...

  20. 19 CFR 206.5 - Public hearing.

    Code of Federal Regulations, 2013 CFR

    2013-04-01

    ... 19 Customs Duties 3 2013-04-01 2013-04-01 false Public hearing. 206.5 Section 206.5 Customs Duties... General § 206.5 Public hearing. (a) Investigations under subpart B of this part. A public hearing on the question of injury and a second public hearing on remedy (if necessary) will be held in connection...

  1. 22 CFR 51.71 - The hearing.

    Code of Federal Regulations, 2014 CFR

    2014-04-01

    ... 22 Foreign Relations 1 2014-04-01 2014-04-01 false The hearing. 51.71 Section 51.71 Foreign... and Revocations § 51.71 The hearing. (a) The Department will name a hearing officer, who will make findings of fact and submit recommendations based on the record of the hearing as defined in § 51.72 to...

  2. 22 CFR 51.71 - The hearing.

    Code of Federal Regulations, 2011 CFR

    2011-04-01

    ... 22 Foreign Relations 1 2011-04-01 2011-04-01 false The hearing. 51.71 Section 51.71 Foreign... and Revocations § 51.71 The hearing. (a) The Department will name a hearing officer, who will make findings of fact and submit recommendations based on the record of the hearing as defined in § 51.72 to...

  3. 22 CFR 51.71 - The hearing.

    Code of Federal Regulations, 2013 CFR

    2013-04-01

    ... 22 Foreign Relations 1 2013-04-01 2013-04-01 false The hearing. 51.71 Section 51.71 Foreign... and Revocations § 51.71 The hearing. (a) The Department will name a hearing officer, who will make findings of fact and submit recommendations based on the record of the hearing as defined in § 51.72 to...

  4. 28 CFR 32.45 - Hearings.

    Code of Federal Regulations, 2011 CFR

    2011-07-01

    ... 28 Judicial Administration 1 2011-07-01 2011-07-01 false Hearings. 32.45 Section 32.45 Judicial... BENEFIT CLAIMS Hearing Officer Determinations § 32.45 Hearings. (a) Except with respect to a remand or referral, at the election of a claimant under subpart B or C of this part, the Hearing Officer shall hold...

  5. 15 CFR 766.13 - Hearings.

    Code of Federal Regulations, 2010 CFR

    2010-01-01

    ... 15 Commerce and Foreign Trade 2 2010-01-01 2010-01-01 false Hearings. 766.13 Section 766.13... PROCEEDINGS § 766.13 Hearings. (a) Scheduling. The administrative law judge, by agreement with the parties or upon notice to all parties of not less than 30 days, will schedule a hearing. All hearings will be...

  6. 28 CFR 4.8 - Hearing.

    Code of Federal Regulations, 2011 CFR

    2011-07-01

    ... 28 Judicial Administration 1 2011-07-01 2011-07-01 false Hearing. 4.8 Section 4.8 Judicial... OF 1974 § 4.8 Hearing. The hearing on the application shall be held at the offices of the Commision in Washington, DC, or elsewhere as the Commission may direct. The hearing shall be held before...

  7. 49 CFR 209.321 - Hearing.

    Code of Federal Regulations, 2011 CFR

    2011-10-01

    ... 49 Transportation 4 2011-10-01 2011-10-01 false Hearing. 209.321 Section 209.321 Transportation... TRANSPORTATION RAILROAD SAFETY ENFORCEMENT PROCEDURES Disqualification Procedures § 209.321 Hearing. (a) Upon receipt of a hearing request complying with § 209.311, an administrative hearing for review of a notice...

  8. 49 CFR 240.409 - Hearings.

    Code of Federal Regulations, 2011 CFR

    2011-10-01

    ... 49 Transportation 4 2011-10-01 2011-10-01 false Hearings. 240.409 Section 240.409 Transportation... Hearings. (a) An administrative hearing for a locomotive engineer qualification petition shall be conducted... conduct of the hearing for the purpose of achieving a prompt and fair determination of all material...

  9. 31 CFR 28.620 - Hearings.

    Code of Federal Regulations, 2011 CFR

    2011-07-01

    ... 31 Money and Finance: Treasury 1 2011-07-01 2011-07-01 false Hearings. 28.620 Section 28.620 Money... EDUCATION PROGRAMS OR ACTIVITIES RECEIVING FEDERAL FINANCIAL ASSISTANCE Procedures § 28.620 Hearings. (a) Opportunity for hearing. Whenever an opportunity for a hearing is required by § 28.615(c), reasonable...

  10. 17 CFR 8.17 - Hearing.

    Code of Federal Regulations, 2010 CFR

    2010-04-01

    ... 17 Commodity and Securities Exchanges 1 2010-04-01 2010-04-01 false Hearing. 8.17 Section 8.17..., SUMMARY, AND MEMBERSHIP DENIAL ACTIONS Disciplinary Procedure § 8.17 Hearing. (a) The following minimum requirements shall apply to any hearing required by this subpart: (1) The hearing shall be fair and shall...

  11. 29 CFR 1650.109 - Hearing.

    Code of Federal Regulations, 2010 CFR

    2010-07-01

    ... 29 Labor 4 2010-07-01 2010-07-01 false Hearing. 1650.109 Section 1650.109 Labor Regulations... Collection of Debts by Salary Offset § 1650.109 Hearing. (a) Request for a hearing. An employee who wants a hearing on the existence of the debt, its amount, or on the proposed offset schedule must send a...

  12. 15 CFR 719.14 - Hearings.

    Code of Federal Regulations, 2010 CFR

    2010-01-01

    ... 15 Commerce and Foreign Trade 2 2010-01-01 2010-01-01 false Hearings. 719.14 Section 719.14... Hearings. (a) Scheduling. Upon receipt of a written and dated request for a hearing, the ALJ shall, by agreement with all the parties or upon notice to all parties of at least 30 days, schedule a hearing....

  13. 28 CFR 4.8 - Hearing.

    Code of Federal Regulations, 2010 CFR

    2010-07-01

    ... 28 Judicial Administration 1 2010-07-01 2010-07-01 false Hearing. 4.8 Section 4.8 Judicial... OF 1974 § 4.8 Hearing. The hearing on the application shall be held at the offices of the Commision in Washington, DC, or elsewhere as the Commission may direct. The hearing shall be held before...

  14. 47 CFR 1.1928 - Hearing.

    Code of Federal Regulations, 2011 CFR

    2011-10-01

    ... 47 Telecommunication 1 2011-10-01 2011-10-01 false Hearing. 1.1928 Section 1.1928... United States Salary Offset-Individual Debt § 1.1928 Hearing. (a) Petition for hearing. (1) An employee may request a hearing by filing a written petition with the Managing Director of the Commission,...

  15. 37 CFR 251.41 - Formal hearings.

    Code of Federal Regulations, 2011 CFR

    2011-07-01

    ... ARBITRATION ROYALTY PANEL RULES AND PROCEDURES COPYRIGHT ARBITRATION ROYALTY PANEL RULES OF PROCEDURE Procedures of Copyright Arbitration Royalty Panels § 251.41 Formal hearings. (a) The formal hearings that... distribution hearings. All parties intending to participate in a hearing of a Copyright Arbitration...

  16. 37 CFR 251.41 - Formal hearings.

    Code of Federal Regulations, 2010 CFR

    2010-07-01

    ... ARBITRATION ROYALTY PANEL RULES AND PROCEDURES COPYRIGHT ARBITRATION ROYALTY PANEL RULES OF PROCEDURE Procedures of Copyright Arbitration Royalty Panels § 251.41 Formal hearings. (a) The formal hearings that... distribution hearings. All parties intending to participate in a hearing of a Copyright Arbitration...

  17. Occupational Hearing Loss in Korea

    PubMed Central

    2010-01-01

    In this article, current status of noise exposure in workplaces, trend of workers with noise-induced hearing loss (NIHL), and prevalence of NIHL in workers by industry and job category in Korea were reviewed. In addition, trends of research on the audiological effects such as hearing loss from noise and occupational hearing loss from non-noise in Korea were addressed through reports in industrial audiology. Though noise exposure level has improved, noise still shows the highest rate of cases exceeding exposure limit among workplace hazards. NIHL is the most common occupational disease except work-related disease such as musculoskeletal disorders and cerebrovascular diseases, and NIHL prevalence is thought to be much higher than reported in official publications. Noise affecting hearing comes from various sources such as workplaces, military settings, areas with exposure to high noise, and specific noise sources. There is also occupational hearing loss by non-noise including chemicals such as organic solvents and heavy metals, barotrauma, and trauma due to welding spark. Noise affects daily life through audiological effects such as hearing loss and tinnitus, non-audiological physical effects (e.g., cardiovascular), and psychosocial and behavioral effects. Development of systematic and comprehensive hearing conservation programs for lowering the noise level in workplaces and preventing the NIHL, and preparation of technological, administrative system for its settlement at workplace are urgently needed. PMID:21258593

  18. Hearing Loss in Palliative Care

    PubMed Central

    Jain, Nelia; Wallhagen, Margaret L.

    2015-01-01

    Abstract Background: Age-related hearing loss is remarkably common, affecting more than 60% of adults over the age of 75. Moreover, hearing loss has detrimental effects on quality of life and communication, outcomes that are central to palliative care. Despite its high prevalence, there is remarkably little written on the impact of hearing loss in the palliative care literature. Objective: The objective was to emphasize its importance and the need for further study. We use a case as a springboard for discussing what is known and unknown about the epidemiology, presentation, screening methodologies, and treatment strategies for age-related hearing loss in palliative care. Discussion: The case describes a 65-year-old man with acute myelogenous leukemia (AML) that has progressed despite treatment. No concerns are raised about communication challenges during conversations between the palliative care team and the patient in his quiet room. However, in the midst of a family meeting, shortly after discussing prognosis, the patient reports that he cannot hear what anyone is saying. Conclusion: We describe simple methods of screening patients for hearing loss, and suggest that practical approaches should be used universally in patient encounters. These include facing the patient, pitching one's voice low, using a pocket talker, and creating a hearing-friendly environment when planning a family or group meeting. PMID:25867966

  19. Hearing loss in shipyard employees

    PubMed Central

    Alexopoulos, Evangelos C.; Tsouvaltzidou, Thomaella

    2015-01-01

    Background: Noise-induced hearing loss (NIHL) is one of the most prevalent occupational illnesses, with a higher incidence in the heavy industry. Objectives of the Study: The aim of this study is to investigate the prevalence of NIHL in Greece and explore its correlations with other job and individual-related factors. Materials and Methods: Questionnaires were administered, and audiograms were conducted to 757 employees of a shipyard company in Greece, both white- and blue-collar, during the period 2006–2009. A modification of the 1979' equation of the American Academy of Otolaryngology was used to calculate hearing loss. Statistical analysis was conducted by means of the SPSS v. 17. Results: A 27.1% of the employees were hearing handicap. Hearing loss was correlated with age, past medical history of ear disease (Meniere's disease, acoustic neuroma, otosclerosis) or injury, hyperlipidemia, job title and level of education. A few questions on subjective hearing ability and symptoms showed strong discriminatory power of hearing pathology. Conclusions: The results of this study emphasize the burden of disease in the shipyard industry, and the need for continuous monitoring, implementation of preventive measures and hearing conservation programs. PMID:26023266

  20. Hearing and underwater noise exposure

    NASA Astrophysics Data System (ADS)

    Smith, P. F.

    1985-08-01

    Exposure of divers to intense noise in water is increasing, yet there is no general hearing conservation standard for such exposures. This paper reviews three theories of underwater hearing as well as empirical data in order to identify some requirements that an underwater conservation standard must meet. Among the problems considered are hearing sensitivity in water, the frequency and dynamic ranges of the water-immersed ear, and nonauditory effects of underwater sound. It is concluded that: first, no well developed theoretical basis exists for extrapolating hearing conservation standards for airborne noise to the underwater situation; second, the empirical data on underwater hearing suggest that the frequency range covered by an underwater hearing conservation standard must be broader than is the case in air; third, in order to establish a general hearing conservation standard for underwater noise exposure further research is required on the dynamic range of the ear in water; fourth, underwater noise exposure may involve hazards to other body systems than the ear; and fifth, some exposure conditions may interfere with job performance of divers.